BioMarin Pharmaceutical Inc. (Nasdaq and SWX: BMRN) today announced financial results for its second quarter ended June 30, 2007. The net loss was $3.9 million ($0.04 per share) for the second quarter of 2007, compared to a net loss of $1.3 million ($0.02 per share) for the second quarter of 2006. The net loss during the second quarter of 2007 includes $4.3 million of non-cash stock compensation expense, compared to $2.1 million of non-cash stock compensation expense during the second quarter of 2006. The results also include Orapred milestone revenue of $4.0 million recognized in the second quarter of 2007, down from the $9.2 million in milestone and license revenue that was recognized in the second quarter of 2006. The reduced Orapred milestone and license revenue contributed to the increased net loss in the second quarter of 2007 as compared to the second quarter of 2006. The net loss for the six months ended June 30, 2007 was $13.2 million ($0.14 per share), compared to a net loss of $11.1 million ($0.14 per share) for the six months ended June 30, 2006. Non-cash stock compensation expense for the six months ended June 30, 2007 and June 30, 2006 was $7.8 million and $3.8 million, respectively.

As of June 30, 2007, BioMarin had cash, cash equivalents, and short-term investments totaling $587.7 million.

"Strong Naglazyme sales for first half of 2007 are driving bottom-line results and fueling the development of our pipeline," said Jean-Jacques Bienaime, Chief Executive Officer of BioMarin. "Our Kuvan program is progressing on track, with three important milestones achieved recently: submission of the NDA filing, initiation of the expanded access program, and two weeks ago, receipt of priority review status. The expanded access program is actively enrolling patients, and we are hopeful that Kuvan will receive FDA approval by the end of November."

Product Sales

Net sales of Naglazyme(R) (galsulfase), an enzyme replacement therapy for mucopolysaccharidosis VI (MPS VI), were $20.9 million for the second quarter of 2007, compared to $18.4 million for the first quarter of 2007, representing a sequential increase of approximately 13.6 percent. Naglazyme net sales for the second quarter of 2006 were $10.3 million. Net sales of Naglazyme for the six months ended June 30, 2007 were $39.3 million, an increase of $22.0 million from net sales of $17.3 million for the six months ended June 30, 2006. BioMarin is commercializing Naglazyme in the United States, Europe, and Latin America, and through distributors in other international markets.

Net sales of Aldurazyme(R) (laronidase), an enzyme replacement therapy for mucopolysaccharidosis I, (MPS I) by BioMarin/Genzyme LLC increased by approximately 23.8 percent to $29.1 million for the second quarter of 2007, compared to $23.5 million in the second quarter of 2006. Aldurazyme net sales for the first quarter of 2007 were $26.8 million. Net sales of Aldurazyme for the six months ended June 30, 2007 were $55.9 million, compared to net sales of $44.9 million for the six months ended June 30, 2006. BioMarin's share of the profit of BioMarin/Genzyme LLC was $6.6 million for the second quarter of 2007, compared to a profit of $4.7 million for the second quarter of 2006. For the six months ended June 30, 2007, BioMarin's share of the profit of BioMarin/Genzyme LLC was $12.7 million, compared to a profit of $8.5 million for the six months ended June 30, 2006.

Royalty and License Revenues

Royalty and license revenues for the second quarter of 2007 were $4.4 million, and include royalties on net product sales of the Orapred product line, including Orapred(R) (prednisolone sodium phosphate oral solution) and Orapred ODT(TM) (prednisolone sodium phosphate orally disintegrating tablets). This also includes a milestone payment of $4.0 million on the first anniversary of FDA approval of Orapred ODT in June 2007. Royalty and license revenues for the second quarter of 2006 were $9.4 million and include a $7.5 million milestone payment related to FDA approval of the marketing application for Orapred ODT. Royalty and license revenues for the six months ended June 30, 2007 were $4.8 million, compared to $9.7 million for the six months ended June 30, 2006.

2007 Financial Guidance Maintained

BioMarin estimates 2007 net sales of Naglazyme to be in the range of $76 million to $82 million. Sales of Aldurazyme by the joint venture for 2007 are expected to be in the range of $115 million to $125 million. BioMarin estimates its GAAP net loss for the fiscal year ending December 31, 2007 to be in the range of $18 million to $23 million. This includes approximately $16 million to $18 million in non-cash stock compensation expense.

  Anticipated Upcoming Milestones
  -- Q307: Initiation of investigator-sponsored pulmonary arterial
           hypertension study
  -- Q407: Receipt of $15 million milestone payment related to Merck Serono
           Kuvan MAA filing
  -- Q407: Decision on the Kuvan NDA by the FDA
  -- Q407: Phenylase IND filing


  Upcoming Investor Conferences
  -- September 10-11: Bear Stearns 20th Annual Healthcare Conference --
     New York City
  -- September 18-20: Merrill Lynch Global Pharma, Biotech, Medtech
     Conference -- London

BioMarin will host a conference call and webcast to discuss second quarter 2007 financial results today, Tuesday, August 7, at 5:00 p.m. ET (23:00 CET). This event can be accessed on the investor section of the BioMarin website at http://www.bmrn.com/.

  Date: August 7, 2007
  Time: 5:00 p.m. ET (23:00 CET)
  U.S. / Canada Dial-in Number:  800.510.9836
  International Dial-in Number:  617.614.3670
  Participant Code: 92042653
  Replay Dial-in Number: 888.286.8010
  Replay International Dial-in Number: 617.801.6888
  Replay Code: 30005812


  About BioMarin

BioMarin develops and commercializes innovative biopharmaceuticals for serious diseases and medical conditions. The company's product portfolio is comprised of two approved products and multiple clinical and preclinical product candidates. Approved products include Naglazyme(R) (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin, and Aldurazyme(R) (laronidase) for mucopolysaccharidosis I (MPS I), a product which BioMarin developed through a 50/50 joint venture with Genzyme Corporation. Investigational product candidates include Kuvan(TM) (sapropterin dihydrochloride), a Phase 3 product candidate for the treatment of phenylketonuria (PKU), and 6R-BH4 for cardiovascular indications, which is currently in Phase 2 clinical development for the treatment of peripheral arterial disease and sickle cell disease. For additional information, please visit http://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 Naglazyme and BioMarin/Genzyme LLC's product Aldurazyme; Sciele Pharma's commercialization of Orapred ODT; the financial performance of the BioMarin as a whole; the timing of BioMarin's clinical trials of 6R-BH4 for other indications; the continued clinical development and commercialization of Aldurazyme, Naglazyme, Kuvan, and 6R-BH4 for other indications; actions by regulatory authorities, including actions related to Kuvan, and 6R-BH4 for other indications; and expectations regarding actions by Merck Serono related to filing the marketing authorization application for Kuvan. 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 the continued commercialization of Naglazyme; our joint venture partner's success in continuing the commercialization of Aldurazyme; Sciele Pharma's success in commercializing Orapred ODT; results and timing of current and planned preclinical studies and clinical trials; our ability to successfully manufacture our products and product candidates; 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 ODT; actual sales of Aldurazyme, Naglazyme and Orapred ODT; Merck Serono's activities related to Kuvan; and those factors detailed in BioMarin's filings with the Securities and Exchange Commission, including, without limitation, the factors contained under the caption "Risk Factors" in BioMarin's 2006 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.

BioMarin(R) and Naglazyme(R) are a registered trademarks of BioMarin Pharmaceutical Inc.

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.

  Contact:

  Investors                       Media
  Eugenia Shen                    Susan Berg
  BioMarin Pharmaceutical Inc.    BioMarin Pharmaceutical Inc.
  (415) 506-6570                  (415) 506-6594

Net Product Sales of BioMarin Pharmaceutical Inc. and BioMarin/Genzyme LLC For the Three and Six Months Ended June 30, 2006 and 2007 (In millions, unaudited)

                                      Three Months Ended   Six Months Ended
                                           June 30,            June 30,
                                         2006      2007      2006     2007
   Aldurazyme (as reported by
    BioMarin/Genzyme LLC) (1)           $23.5     $29.1     $44.9    $55.9
   Naglazyme                             10.3      20.9      17.3     39.3


   (1) The Company recognizes its 50% share of the net income of
       BioMarin/Genzyme LLC as Equity in the 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 Six Months Ended, June 30, 2006 and 2007
           (In thousands, except for per share data, unaudited)

                              Three Months Ended      Six Months Ended
                                   June 30,                June 30,
                               2006        2007        2006       2007

  Revenues:
    Net product sales         $9,657     $20,941     $18,636    $39,276
    Collaborative agreement
     revenues                  4,435       3,505       8,949      7,652
    Royalty and license
     revenues                  9,358       4,438       9,677      4,795
      Total revenues          23,450      28,884      37,262     51,723
  Operating expenses:
    Cost of sales (excludes
     amortization of
     developed product
     technology)                 789       4,557       2,512      8,674
    Research and
     development              15,779      19,186      28,058     37,345
    Selling, general
     and administrative       11,871      17,649      22,767     33,935
    Amortization of
     acquired intangible
     assets                    1,093       1,093       1,466      2,185
      Total operating
       expenses               29,532      42,485      54,803     82,139
  Loss from operations        (6,082)    (13,601)    (17,541)   (30,416)
  Equity in the income of
   BioMarin/Genzyme LLC        4,745       6,550       8,545     12,713
  Interest income              4,034       6,907       4,736     10,601
  Interest expense            (4,022)     (3,720)     (6,846)    (6,055)
    Net loss                 $(1,325)    $(3,864)   $(11,106)  $(13,157)
    Net loss per share,
     basic and diluted        $(0.02)     $(0.04)     $(0.14)    $(0.14)
  Weighted average common
   shares outstanding,
   basic and diluted          85,341      95,796      80,181     95,180



              BIOMARIN PHARMACEUTICAL INC. AND SUBSIDIARIES

                       CONSOLIDATED BALANCE SHEETS
                   December 31, 2006 and June 30, 2007
           (In thousands, except for share and per share data)

                                               December 31,     June 30,
                                                   2006           2007
                                                               (unaudited)
                        ASSETS
  Current assets:
    Cash and cash equivalents                      $89,162       $205,010
    Short-term investments                         199,685        382,700
    Accounts receivable, net                        14,670         15,900
    Advances to BioMarin/Genzyme LLC                 1,596          1,302
    Inventory                                       25,075         30,714
    Other current assets                             4,036          5,306
      Total current assets                         334,224        640,932
  Investment in BioMarin/Genzyme LLC                31,457         33,269
  Property, plant and equipment, net                55,466         58,780
  Acquired intangible assets, net                   11,655          9,470
  Goodwill                                          21,262         21,262
  Restricted cash                                    1,731          3,103
  Other assets                                       7,641         14,823
      Total assets                                $463,436       $781,639


          LIABILITIES AND STOCKHOLDERS' EQUITY
  Current liabilities:
    Accounts payable and accrued liabilities       $32,166        $30,604
    Current portion of acquisition
     obligation, net of discount                     6,787          6,785
    Current portion of deferred revenue              7,092          7,154
      Total current liabilities                     46,045         44,543
  Convertible debt                                 223,940        497,375
  Long-term portion of acquisition
   obligation, net of discount                      68,548         67,332
  Deferred revenue, net of current portion           5,023          1,509
  Other long-term liabilities                        2,078          3,278
      Total liabilities                            345,634        614,037
  Stockholders' equity:
    Common stock, $0.001 par value:
     150,000,000 and 250,000,000 shares
     authorized at December 31, 2006 and
     June 30, 2007, respectively; 91,725,528
     and 95,901,593 shares issued and
     outstanding at December 31, 2006 and
     June 30, 2007, respectively                        92             96
    Additional paid-in capital                     709,359        772,297
    Accumulated other comprehensive loss               (25)            (7)
    Accumulated deficit                           (591,624)      (604,784)
      Total stockholders' equity                   117,802        167,602
      Total liabilities and
       stockholders' equity                       $463,436       $781,639

First Call Analyst:
FCMN Contact: eshen@bmrn.com

SOURCE: BioMarin Pharmaceutical Inc.