A twenty-four year old white man with Ehlers-Danlos syndrome (Type IV) is presented herein. He demonstrates, or has demonstrated, spontaneous bowel perforation, club feet, cryptorchidism, spontaneous vascular rupture, prominent veins, and distal joint hypermobility. In addition, laparotomy for an acute abdominal condition at four years of age revealed a persisting and gangrenous vitelline duct cyst. We believe this is the first report of this congenital anomaly associated with this syndrome.