MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, January 30, 2019 – Orphazyme A/S, 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 Novo Holdings A/S on behalf of Novo Nordisk Fonden that Novo Holdings A/S as of today’s date holds a total of 2,984,368 shares in the Company, corresponding to 14.97% of the share capital and the voting rights in the Company.

Novo Holdings A/S is wholly-owned by Novo Nordisk Fonden.