BioMarin Announces Third Quarter 2005 Financial Results

November 1, 2005

Financial Guidance for 2005 Improved
Conference Call and Webcast to Be Held Wednesday, November 2 at Noon ET (18:00 CET)
Nov 1, 2005
PRNewswire-FirstCall
NOVATO, Calif.

BioMarin Pharmaceutical Inc. (Nasdaq and SWX: BMRN) today announced results for its third quarter ended September 30, 2005. The net loss was $15.5 million ($0.21 per share) for the third quarter of 2005 compared to $29.5 million ($0.46 per share) for the third quarter of 2004. The net loss was $59.3 million ($0.88 per share) for the nine months ended September 30, 2005, compared to $105.0 million ($1.63 per share) for the nine months ended September 30, 2004.

"We are pleased with the continued sales and profitability growth of Aldurazyme through the BioMarin/Genzyme LLC joint venture and with the progress we have made recently in getting MPS VI patients onto commercial therapy with Naglazyme," stated Jean-Jacques Bienaime, Chief Executive Officer of BioMarin. "We have improved our financial guidance for 2005 and remain committed to managing our resources judiciously as we continue to advance our product pipeline." Mr. Bienaime continued, "Enrollment in the Phase 3 trial of Phenoptin for PKU is going well and we remain on track to announce data from the double-blind portion of the trial in late March of 2006."

Net sales of Aldurazyme(R) (laronidase), an enzyme replacement therapy for mucopolysaccharidosis I, (MPS I) by BioMarin/Genzyme LLC increased 95 percent to $20.1 million for the third quarter of 2005, compared to $10.3 million in the third quarter of 2004. Net sales for the nine months ended September 30, 2005 were $55.2 million compared to $27.0 million for the same period in 2004, representing an increase of 104 percent. BioMarin's share of the profit from BioMarin/Genzyme LLC was $3.4 million for the third quarter of 2005, compared to a loss of $0.5 million for the third quarter of 2004. BioMarin's share of the profit from BioMarin/Genzyme LLC for the nine months ended September 30, 2005, was $8.8 million, compared to a loss of $4.0 million for the nine months ended September 30, 2004.

Net sales of Naglazyme(TM) (galsulfase), an enzyme replacement therapy for mucopolysaccharidosis VI (MPS VI), were $2.3 million for the third quarter of 2005, and $2.4 million for the nine months ended September 30, 2005. Naglazyme was approved by the U.S. Food and Drug Administration (FDA) on May 31, 2005 and launched by BioMarin's U.S.-based sales force on June 20, 2005. The FDA has designated Naglazyme an orphan drug, which grants it seven years of market exclusivity in the United States. In mid-September 2005, the scientific committee of the European Medicines Agency (EMEA) issued a positive opinion on the Marketing Authorization Application (MAA) for Naglazyme. BioMarin plans to commercialize Naglazyme in the European Union on its own with a small and efficient E.U.-based sales force, pending final approval by the European Commission.

Net sales of Orapred(R) (prednisolone sodium phosphate oral solution), including the branded and authorized generic products, of $0.2 million for the third quarter of 2005 were flat compared to net sales in the same period of 2004. Orapred net sales recorded by BioMarin for the nine months ended September 30, 2005 were $6.5 million compared to $4.7 million for the same period in 2004. BioMarin began recording sales of Orapred in May 2004 at the time the product was acquired.

As of September 30, 2005, BioMarin had cash, cash equivalents, short-term investments and cash balances related to long-term debt of approximately $83.9 million.

  Financial Guidance

  2005 Projected Net Product Sales

BioMarin has revised its 2005 product revenue guidance for the fiscal year ending December 31, 2005:

   --   Aldurazyme for MPS I:  A range of $74 million to $77 million
        (increased from a range of $70 million to $75 million)
   --   Naglazyme for MPS VI:  A range of $4 million to $6 million
        (unchanged)
   --   Orapred for inflammatory conditions:  A range of $7 million to $8
        million (decreased from a range of $8 million to $10 million)

  2005 Projected Net Loss and End of Year Cash Balance

BioMarin has revised its 2005 financial guidance for projected net loss and end of year cash balance for the fiscal year ending December 31, 2005:

   --   GAAP net loss: A range of $73 million to $78 million, which includes
        $6.7 million of Orapred deal-related expenses (decreased from a
        range of $75 million to $80 million)
   --   End of year cash balance:  A range of $59 million to $64 million in
        cash, cash equivalents, short-term investments and cash balances
        related to long-term debt (increased from a range of $55 million to
        $60 million)

  Recent Events and Third Quarter 2005 Highlights

   --   On September 15, BioMarin announced that it had received a positive
        opinion from the EMEA on the MAA for Naglazyme for MPS VI.
   --   On August 22, Brazilian regulatory authorities approved Aldurazyme
        for the treatment of MPS  I.
   --   On October 19, BioMarin announced that the FDA had accepted for
        filing the New Drug Application for Orapred ODT(TM) (prednisolone
        sodium phosphate orally disintegrating tablets) for the treatment of
        inflammatory conditions.  The company expects to receive a response
        from the FDA by mid-2006.
   --   On August 23, BioMarin announced the promotion of Jeffrey Cooper to
        the position of Chief Financial Officer.  Mr. Cooper succeeds Louis
        Drapeau who retired at the end of October 2005.

  Upcoming Company Events

On Tuesday, December 6, 2005, BioMarin will host a "Research and Development Day" to discuss the company's products and product pipeline. The event, which will be held at the Four Seasons Hotel located in New York City, New York, will take place from 7:30 a.m. to 10:30 a.m. ET. Interested parties can access a live audio web cast of the event via the investor section of the BioMarin website, www.BMRN.com.

Upcoming Company Milestones

BioMarin expects to continue to advance its product and clinical-stage product portfolio in the coming months. The following is a list of the company's projected near-term milestones:

   --   Announce the outcome of the European Commission's review of the MAA
        for Naglazyme (first quarter of 2006)
   --   Announce data from the Phase 3 clinical trial of Phenoptin(TM)
        (sapropterin dihydrochloride) for the treatment of PKU (late March
        of 2006)

BioMarin will host a conference call and webcast to discuss third quarter financial results Wednesday, November 2, at Noon ET (18:00 CET). This event can be accessed on the investor section of the BioMarin website at www.BMRN.com.

   Date: November 2, 2005
   Time: Noon ET (18:00 CET)
   U.S. & Canada Toll-free Dial in #: 866.700.0133
   International Dial in #: 617.213.8831
   Participant Code:  78103268
   Replay Toll-free Dial in #: 888.286.8010
   Replay International Dial in #: 617.801.6888
   Replay Code: 32265695

  About BioMarin

BioMarin develops and commercializes innovative biopharmaceuticals for serious diseases and medical conditions. The company's product portfolio is comprised of three approved products and multiple clinical and preclinical product candidates. Approved products include Naglazyme(TM) (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin, Aldurazyme(R) (laronidase) for mucopolysaccharidosis I (MPS I), and Orapred(R) (prednisolone sodium phosphate oral solution) for inflammatory conditions. Investigational product candidates include Phenoptin(TM) (sapropterin dihydrochloride), a Phase 3 product candidate for the treatment of phenylketonuria (PKU). For additional information, please visit www.BMRN.com.

Information on BioMarin's website, www.BMRN.com, is not incorporated by reference into this press release.

Forward-Looking Statement

This press release contains forward-looking statements about the business prospects of BioMarin Pharmaceutical Inc., including, without limitation, statements about: the sales expectations of BioMarin's products Naglazyme and Orapred (including its branded and authorized generic products) and BioMarin/Genzyme LLC's product Aldurazyme; the financial performance of the BioMarin as a whole; the timing of BioMarin's clinical trials of Phenoptin; the continued clinical development and commercialization of Aldurazyme, Naglazyme, Orapred, and Phenoptin; and actions by regulatory authorities, including actions related to Naglazyme, Orapred and Phenoptin. These forward- looking statements are predictions and involve risks and uncertainties such that actual results may differ materially from these statements. These risks and uncertainties include, among others: our success in continuing commercialization of Naglazyme and Orapred; our joint venture partner's success in continuing the commercialization of Aldurazyme; results and timing of current and planned preclinical studies and clinical trials, including the Phase 3 clinical trial of Phenoptin; the content and timing of decisions by the U.S. Food and Drug Administration, the European Commission and other regulatory authorities concerning each of the described products and product candidates; the market for each of these products and particularly Aldurazyme, Naglazyme and Orapred; actual sales of Aldurazyme, Naglazyme and Orapred; and those factors detailed in BioMarin's filings with the Securities and Exchange Commission, including, without limitation, the factors contained under the caption "Factors That May Affect Future Results" in BioMarin's 2004 Annual Report on Form 10-K, as amended, and the factors contained in BioMarin's reports on Form 10-Q and Form 8-K. Stockholders are urged not to place undue reliance on forward-looking statements, which speak only as of the date hereof. BioMarin is under no obligation, and expressly disclaims any obligation to update or alter any forward-looking statement, whether as a result of new information, future events or otherwise.

NOTE: Aldurazyme(R) is a registered trademark of BioMarin/Genzyme LLC.

Orapred(R) is a registered trademark of Medicis Pediatrics, Inc. and is used under license.

   Contacts:

   Investors                          Media
   Joshua A. Grass                    Susan Ferris
   BioMarin Pharmaceutical Inc.       BioMarin Pharmaceutical Inc.
   415-506-6777                       415-506-6701


          NET PRODUCT SALES OF BIOMARIN AND BIOMARIN/GENZYME LLC
     For the Three and Nine Months Ended September 30, 2004 and 2005
                         (in millions, unaudited)

                           Three Months Ended          Nine Months Ended
                              September 30,              September 30,
                            2004         2005          2004         2005

  Aldurazyme (as reported
   by Bio/Marin/Genzyme
   LLC) (1)                $10.3        $20.1        $27.0        $55.2
  Orapred                    0.2          0.2          4.7          6.5
  Naglazyme                   --          2.3           --          2.4

(1) The company recognizes its 50% share of the net income/loss of BioMarin/Genzyme LLC as Equity in the loss/(income) of BioMarin/Genzyme LLC in the company's consolidated statements of operations.

              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                  CONSOLIDATED STATEMENTS OF OPERATIONS
     For the Three and Nine Months Ended September 30, 2004 and 2005
           (In thousands, except for per share data, unaudited)

                           Three Months Ended         Nine Months Ended
                              September 30,             September 30,
                            2004        2005          2004         2005

  Revenues:
   Net product sales        $181       $2,456       $4,744       $8,881
   Collaborative agreement
    revenues                  --        5,123           --        7,313

  Total revenues             181        7,579        4,744       16,194
  Operating expenses:
   Cost of sales (excludes
    amortization of developed
    product technology)       35          164          648        1,301
   Research and
    development           11,806       13,894       37,670       43,708
   Selling, general and
    administrative        12,761        9,797       24,304       30,480
   Amortization of
    acquired intangible
    assets                 1,694          286        2,513          858
   Acquired in-process
    research and
    development               --           --       35,444           --

     Total operating
      expenses            26,296       24,141      100,579       76,347

  Equity in the loss/
   (income) of BioMarin/
   Genzyme LLC               507      (3,388)        3,965      (8,766)

  Loss from operations  (26,622)     (13,174)     (99,800)     (51,387)

  Interest income            573          561        1,997        1,177
  Interest expense       (3,429)      (2,863)      (7,218)      (9,064)

   Net loss            $(29,478)    $(15,476)   $(105,021)    $(59,274)

   Net loss per share,
    basic and diluted    $(0.46)      $(0.21)      $(1.63)      $(0.88)

  Weighted average common
   shares outstanding,
   basic and diluted      64,384       71,996       64,316       67,047



              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                       CONSOLIDATED BALANCE SHEETS
           (In thousands, except for share and per share data)

                                               December 31,  September 30,
                                                 2004(1)         2005
                                                              (unaudited)
                           ASSETS
  Current assets:
      Cash and cash equivalents                    $13,081        $56,213
      Short-term investments                        35,734          9,700
      Restricted cash                               25,180             --
      Accounts receivable, net                       4,047          5,328
      Advances to BioMarin/Genzyme LLC               2,160            852
      Inventory                                      2,316          7,720
      Other current assets                           2,641          2,375

           Total current assets                     85,159         82,188
  Cash balances related to long-term debt           16,406         18,014
  Investment in BioMarin/Genzyme LLC                23,129         30,411
  Property and equipment, net                       42,501         37,799
  Acquired intangible assets, net                   16,451         15,592
  Goodwill                                          45,053         21,262
  Other assets                                       4,267          3,576

           Total assets                           $232,966       $208,842

            LIABILITIES AND STOCKHOLDERS' DEFICIT
  Current liabilities:
      Accounts payable and accrued liabilities     $27,249        $22,936
      Current portion of acquisition obligation,
       net of discount                              39,122          7,812
      Current portion of equipment and facility
       loans                                         3,683          3,884
      Current portion of deferred revenue               --          9,592

           Total current liabilities                70,054         44,224
  Convertible debt                                 125,000        125,000
  Long-term portion of acquisition obligation,
   net of discount                                  86,632         71,433
  Equipment and facility loan, net of current
   portion                                          16,406         18,014
  Deferred revenue, net of current portion              --         12,500
  Other long-term liabilities                        2,852          3,050

           Total liabilities                       300,944        274,221

  Stockholders' deficit:
      Common stock, $0.001 par value:
       150,000,000 shares authorized; 64,501,159
       and 73,860,139 shares issued and outstanding
       at December 31, 2004 and September 30, 2005,
       respectively                                     65             74
      Additional paid-in capital                   421,141        482,658
      Accumulated other comprehensive loss           (363)           (16)
      Accumulated deficit                        (488,821)      (548,095)

           Total stockholders' deficit            (67,978)       (65,379)

           Total liabilities and
            stockholders' deficit                 $232,966       $208,842

   (1)  December 31, 2004 balances were derived from the audited
        consolidated financial statements.

SOURCE: BioMarin Pharmaceutical Inc.

CONTACT: investors, Joshua A. Grass, +1-415-506-6777, or media, Susan
Ferris, +1-415-506-6701, or BioMarin Pharmaceutical Inc.

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