Our patient presented with ruptured chordae tendinea, spontaneous hemothorax, recurrent intermittent hemoptysis, and skin findings that were compatible with type 4, or vascular-type, EDS. Type 4 EDS is a rare, but serious, inheritable connective tissue disorder, carrying the worst prognosis of all subtypes of the syndrome. Reported prevalence ranges from one in 100,000 to one in 1,000,000, accounting for 5% to 10% of all EDS cases. In general, patients with type 4 EDS do not present with the joint hypermobility seen in other subtypes. Physical examination findings suggesting this diagnosis include skin hyperelasticity, serpiginous rash, extensive bruising, and facial findings such as pinched nose, low-set ears, and creasing of the nasolabial folds (acrogeria). Although arterial rupture is the most common cause of death in type 4 EDS, perforation of viscous organs as well as serious respiratory manifestations carry a high mortality. A study of 419 patients with EDS estimated the median survival to be 48 years.