The Ehlers-Danlos syndrome type IV is a heritable connective-tissue disorder characterized by thin translucent skin, pronounced bruising and scarring, and extreme tissue fragility. Obstetrical complications include premature rupture of membranes, rupture of blood vessels and gravid uterus, tearing of perineum, vagina, urethra and bladder, requesting specific prophylactic and therapeutic measurements. Hereditary transmission is usually autosomal dominant with variable expression. Histological examination of skin biopsy and biochemical analysis of collagen proteins from skin fibroblast cultures confirm the clinical diagnosis. DNA studies offer the possibility of prenatal diagnosis in suitable families. The consecutive severe obstetrical complications of a woman with Ehlers-Danlos syndrome type IV are reported here. The patient died at age 33 years from renal artery rupture.