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Hepatobiliary fibropolycystic disease is cause of hepatosplenomegaly & portal hypertension. This disease entities was composed of polycystic liver disease, congenital hepatic fibrosis, choledochal cyst, Caroli’s disease(communicating, cavernous biliary ectasia), & von Meyenburg’s complex. Clinical symptoms are space occupying lesion, portal hypertension, & cholangitis. The pathogenesis of hepatobiliary fibropolycystic disease is unknown. Two theories has been suggested. In one, the basic defect is attributable to malformation of the embryonal ductal biliary plates. In the other theory, cyst formation occurs because of an abnormal pancreaticobiliary junction with resultant chronic pancreatic reflux into the biliary tree. We report a case of fibropolycystic disease with review of the literature.
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