Company announcement                                                                                        
Orphazyme A/S
No. 06/2023                                                                                                            
Ole Maaløes Vej 3                                                                                                                
DK-2200 Copenhagen N
                                                                                                                                                      
www.orphazyme.com
Company Registration No. 32266355

Copenhagen, Denmark, May 12, 2023 – Orphazyme A/S (ORPHA.CO) (the “Company”), hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Scandinavian Investment Group A/S that Scandinavian Investment Group A/S holds shares, corresponding to more than 5% of the total share capital and voting rights in the Company.

The Company has today become aware that each of Scandinavian Investment Group A/S, MH Investment ApS and Nordic Compound Invest A/S hold slightly below 10% of the total share capital and voting rights in the Company, see also company announcements no. 4 and 5 of 9 May 2023.

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, Chief Executive Officer and Chief Financial Officer: +45 28 98 90 55