A 30-year-old Chinese man with vascular type Ehlers-Danlos Syndrome presents with spontaneous right forearm compartment syndrome due to pseudoaneurysms along the radial artery. Emergency fasciotomy and reconstruction of the radial artery with a saphenous vein graft were performed. Genetic test showed a heterozygous DNA change c. 1852 G > C in COL3A1 gene.