Splenic rupture secondary to V-EDS has been reported rarely in the literature but never in a young child.2Vascular, or type 4, EDS is an autosomal dominant connective tissue disorder caused by a mutation in type III procollagen (COL3A1) and carries the highest mortality rate of the EDS subtypes.1, 3, 4, 5It is associated with translucent skin, easy bruising, and an increased risk of arterial, intestinal, and solid organ rupture. The gene discussed is COL3A1; the disease is Ehlers-Danlos syndrome.