Vascular Ehlers-Danlos syndrome (vEDS), also known as EDS type IV, is characterized by translucent skin, joint hypermobility, and a high risk of arterial rupture.[1] The incidence of vEDS is approximately one in 150,000.[2] COL3A1 gene heterozygous mutations can cause vEDS, which encodes the pro-α1 (III) chain of type III procollagen. The gene discussed is COL3A1; the disease is Ehlers-Danlos syndrome, vascular type.