Ehlers-Danlos syndrome is a genetically transmitted disorder of connective tissue characterized by hyperelasticity of the skin, hyperflexibility and looseness of the joints, easy bruisability of the skin, and in the more severe forms of the disease, arterial fragility and tendency to rupture. Pregnant patients with Ehlers-Danlos syndrome are at increased risk for various complications, most serious of which is rupture of a major vessel. The present case report is the second known report of a maternal death from rupture of a major artery in a patient with the severe type IV variant of the syndrome. The advisability of pregnancy and genetic counseling are discussed.