Esophagogastric variceal bleeding as a debut of Caroli's syndrome

Caroli's disease is a congenital disease characterized by saccular or cystic dilatations of intrahepatic bile ducts. It is called Caroli's syndrome (CS) when intrahepatic dilations are associated with congenital hepatic fibrosis. CS is an infrequent cause of gastrointestinal bleeding in young people that should be considered when saccular dilatations of the bile duct are associated with portal hypertension (PHT). We present a rare case with gastrointestinal bleeding as first manifestation of CS: A 13-year-old woman with no relevant medical history was admitted to the hospital with an episode of hematemesis.