MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, April 17, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Danske Bank A/S that Danske Bank as of April 16, 2019 holds a total 1,000,395 shares in the Company, corresponding to 5.00% of the share capital and 7.69% of the voting rights in the Company.

Danske Bank A/S’ shareholding consists of a 4.88% indirect and a 0.12% direct ownership through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest, and Danske Invest SICAV. Danske Bank A/S’ voting rights in the Company consists of a 7.57% indirect and a 0.12% direct control through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest, and Danske Invest SICAV.