Aim: Vascular Ehlers-Danlos Syndrome (vEDS) is an autosomal- dominant inherited disorder result on collagen type III alpha-1 chain (COL3A1) gene mutation. vEDS is associated with a decreased life expectancy due to spontaneous arterial, intestinal, and uterine rupture. The diagnosis of vEDS is supported by genetic testing confirming the presence of pathogenic variations in COL3A1. Although how COL3A1 mutation in the precise mechanism can lead to the multiple vascular involvements seen at a pathological level remains unclear. COL3A1 encodes the Collagen alpha-1(III) chain in humans. The alterations in content and properties of type III collagen, can lead to organ fragility. Genome sequencing revealed heterozygous COL3A1 variants (c.4223C>T p.F1408S ) as likely genetic cause of vEDS in this present case. Methods: We assessed the young adult diagnosed with Stanford A aortic dissection without a history of hypertension and undertook Sanger sequencing. Results: We identified novel compound heterozygous variant in COL3A1: a missense- c.4223C>T p.F1408S Conclusion: Given the clinical phenotype and identified variants we suggest that this is the first patient reported to date with vEDS due to c.4223C>T mutations in COL3A1.