Authors report an uncommon case of spontaneous rupture of common iliac artery in a man 43 years old with Ehlers-Danlos syndrome. The clinical presentation was devious in the beginning and catastrophic after few hours with a haemo-peritoneum and haemorragic shock. The Ehlers-Danlos syndrome is a rare affection of the connective tissue with an incidence of 1/5000, representing one of the most common disorders of the connective tissue. This disease is characterized by the fragility of arteries, intestine and uterus. Its presentation is often catastrophic, with rupture of a big artery, rupture of uterus during pregnancy or bowel perforation. The mean age of death in subjects with Ehlers-Danlos syndrome is 45 years. This syndrome is inherited in most cases in an autosomal dominant manner; 50% of the cases are due to new mutations. A minority of cases, due to deficit of tenascina X, is inherited in an autosomal recessive manner.