BioMarin Pharmaceutical Inc. (Nasdaq and SWX: BMRN) today announced results for its first quarter ended March 31, 2005. The net loss was $22.5 million ($0.35 per share) for the first quarter of 2005 compared to $19.9 million ($0.31 per share) for the first quarter of 2004.

"Our recent accomplishments, namely the initiation of the Phase 3 clinical trial of Phenoptin, and the expansion of our agreement with Daiichi Suntory relating to 6R-BH4 for use in treating vascular dysfunction in diabetes and cardiovascular diseases, reflect BioMarin's strategy to aggressively advance its clinical programs and to identify product opportunities that draw upon our existing expertise," stated Louis Drapeau, acting Chief Executive Officer of BioMarin. "In anticipation of a favorable outcome from the FDA's review of the marketing application for rhASB for the treatment of MPS VI, we are focused on doing all that is required to ensure the successful launch of rhASB, pending regulatory approval."

Net sales of Aldurazyme(R) (laronidase) by BioMarin/Genzyme LLC were $15.9 million for the first quarter of 2005, compared with $7.4 million in the first quarter a year ago. BioMarin's 50 percent share of the BioMarin/Genzyme LLC was a $2.1 million profit for the first quarter of 2005, compared to a loss of $1.8 million for the first quarter in 2004. This increase is due primarily to increasing sales of Aldurazyme as additional patients are identified and as patients are transitioned from extension studies onto commercial therapy.

Net sales of Orapred(R) (prednisolone sodium phosphate oral solution), including its branded and authorized generic products, in the first quarter of 2005 were $5.0 million. BioMarin began recording sales of Orapred in May 2004 at the time of its acquisition.

BioMarin's net loss in the first quarter of 2005 included: $15.0 million in ongoing research and development expenses primarily related to rhASB (galsulfase) for mucopolysaccharidosis VI (MPS VI), Phenoptin(TM) (sapropterin hydrochloride) for phenylketonuria (PKU), and Orapred oral disintegrating tablet ('Orapred ODT') for severe asthma; and $10.6 million in selling, general and administrative expenses.

Additionally, in January 2005, BioMarin reached a settlement with Medicis totaling $27.0 million, which reflects a reduction of the acquisition liability and goodwill. As part of the settlement, BioMarin expects to receive payments totaling $6.0 million in 2005 for product returns of which $2.0 million was received in the first quarter of 2005.

As of March 31, 2005, BioMarin had cash, cash equivalents, short-term investments, restricted cash and cash balances related to long-term debt of approximately $62.3 million.

Upcoming Company Milestones

BioMarin expects to continue to advance its 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 U.S. Food and Drug Administration's (FDA) review of the Biologics License Application for rhASB for the treatment of MPS VI. Pursuant to the Prescription Drug User Fee Act (PDUFA), the FDA will take action by May 31, 2005;

-- Announce the outcome of the European Medicines Agency's review of the Marketing Authorization Application for rhASB for the treatment of MPS VI in the second half of 2005; and

-- Announce data from the Phase 3 clinical trial of Phenoptin for the treatment of PKU in the fourth quarter of 2005.

Upcoming Corporate Presentations

BioMarin will present an overview of its business and product development programs at the following health care and biotechnology investment conferences:

-- Pacific Growth Equities Life Sciences Growth Conference, June 6-8, 2005, San Francisco, California

-- Morgan Stanley Small Cap Executive Conference, June 13-15, 2005, New York City, New York

These corporate presentations will be available via webcast on the investor section of the BioMarin corporate website, www.BMRN.com.

BioMarin will host a conference call and webcast to discuss first quarter financial results Monday, May 16, at 12:00 PM EDT (18:00 CEST). This event can be accessed on the investor section of the BioMarin website at www.BMRN.com.

   Date:  May 16, 2005
   Time:  12:00 PM EDT (18:00 CEST)
   U.S. & Canada Toll-free Dial in #:  800-299-9086
   International Dial in #:  617-786-2903
   Participant Code:  69323515
   Replay Toll-free Dial in #:  888-286-8010
   Replay International Dial in #:  617-801-6888
   Replay Code: 30154746

BioMarin develops and commercializes innovative biopharmaceuticals for serious diseases and medical conditions. The company's product portfolio is comprised of two approved products, Aldurazyme(R) (laronidase) for mucopolysaccharidosis I (MPS I) and Orapred(R) (prednisolone sodium phosphate oral solution) for severe asthma, and multiple investigational product candidates including rhASB (galsulfase), a BLA/MAA-stage product candidate under review for the treatment of mucopolysaccharidosis VI (MPS VI), and Phenoptin(TM) (sapropterin hydrochloride), a Phase 3 product candidate for the treatment of phenylketonuria (PKU). For additional information, please visit www.BMRN.com. Information on BioMarin's website 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 product Orapred (including its branded and authorized generic products) and BioMarin/Genzyme LLC's product Aldurazyme; the financial performance of the Company as a whole; the timing of BioMarin's clinical trials of Phenoptin; the continued clinical development and commercialization of Aldurazyme, Orapred, rhASB and Phenoptin; and actions by regulatory authorities, including actions related to rhASB. 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 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; the market for each of these products and particularly Aldurazyme and Orapred; actual sales of Aldurazyme and Orapred; product returns of Orapred; the possible development of competing products; the effect on sales of Orapred following the recent approval of a generic product that is therapeutically equivalent to Orapred; the implementation of a settlement with Medicis; 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 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


              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

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

                                                 December 31,     March 31,
                                                   2004 (1)         2005
                                                                (unaudited)

                           ASSETS
  Current assets:
      Cash and cash equivalents                    $13,081        $11,246
      Short-term investments                        35,734         18,513
      Restricted cash                               25,298         12,930
      Accounts receivable, net                       4,047          1,831
      Advances to BioMarin/Genzyme LLC               2,160            604
      Inventory                                      2,316          2,439
      Other current assets                           2,641          6,036

        Total current assets                        85,277         53,599

  Cash balances related to long-term debt           16,406         19,603
  Investment in BioMarin/Genzyme LLC                23,129         25,217
  Property and equipment, net                       42,501         41,179
  Acquired intangible assets, net                   16,451         16,165
  Goodwill                                          45,053         21,262
  Other assets                                       4,149          3,908

        Total assets                              $232,966       $180,933

         LIABILITIES AND STOCKHOLDERS' DEFICIT
  Current liabilities:
      Accounts payable and accrued liabilities     $27,249        $25,639
      Current portion of acquisition obligation,
       net of discount                              39,122         20,946
      Current portion of equipment and
       facility loans                                3,683          4,110

        Total current liabilities                   70,054         50,695

  Convertible debt                                 125,000        125,000
  Equipment and facility loan, net of
   current portion                                  16,406         19,603
  Long-term portion of acquisition obligation,
   net of discount                                  86,632         72,821
  Other long-term liabilities                        2,852          3,049

        Total liabilities                          300,944        271,168

  Stockholders' deficit:
      Common stock, $0.001 par value:
       150,000,000 shares authorized;
       64,501,159 and 64,511,159 shares
       issued and outstanding at
       December 31, 2004 and March 31, 2005,
       respectively                                     65             65
      Additional paid-in capital                   421,141        421,214
      Accumulated other comprehensive loss            (363)          (235)
      Accumulated deficit                         (488,821)      (511,279)

        Total stockholders' deficit                (67,978)       (90,235)

        Total liabilities and stockholders'
         deficit                                  $232,966       $180,933

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


              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                  CONSOLIDATED STATEMENTS OF OPERATIONS

           For the Three Months Ended, March 31, 2004 and 2005

           (In thousands, except for per share data, unaudited)

                                                     Three Months Ended
                                                           March 31,

                                                      2004          2005
  Net product sales                                    $--         $4,989

  Operating expenses:
      Cost of sales (excludes amortization
       of developed product technology)                 --            660
      Research and development                      13,695         14,992
      Selling, general and administrative            3,881         10,567
      Amortization of acquired intangible assets        --            286
      Equity in the loss/(income) of
       BioMarin/Genzyme LLC                          1,759         (2,076)

        Total operating expenses                    19,335         24,429

  Loss from operations                             (19,335)       (19,440)
  Interest income                                      761            241
  Interest expense                                  (1,371)        (3,259)

      Net loss                                    $(19,945)      $(22,458)

      Net loss per share, basic and diluted         $(0.31)        $(0.35)

  Weighted average common shares outstanding,
   basic and diluted                                64,225         64,511

SOURCE: BioMarin Pharmaceutical Inc.