We report a case of a 13-year-old girl who presented with acute abdomi
nal pain secondary to a sigmoid colon perforation, History, physical e
xamination, and laboratory and radiographic studies were all suggestiv
e, though not diagnostic, of an abdominal catastrophe, Her father died
at the age of 30 from complications of bowel perforations and a vascu
lar aneurysm, The unusual operative findings in our patient, together
with her father's medical history, Lead to the underlying diagnosis of
Ehlers-Danlos syndrome, Knowledge of this family history at the time
of presentation could have aided in diagnosis, Clinical manifestations
and etiology of Ehlers-Danlos syndrome are discussed.