[Fibropolycystic diseases of the liver and bile ducts--a case report and review of the literature].

: A case of congenital cystic dilatation of the intrahepatic bile ducts (Caroli's disease) is reported. This 20-year-old patient presented also with congenital hepatic fibrosis, fusiform dilatation of the common bile duct and cysts of the liver parenchyma, accompanied by medullary sponge kidneys. The dominant clinical feature was recurrent septic cholangitis. Clinical picture, as well as the diagnostic and therapeutic problems of the individual entities of hepatobiliary fibropolycystic disease are discussed. Although these belong to a family of closely related malformations, whereby involvement of different anatomic levels varies from case to case, such a complex combination as in this patient has not, to our knowledge, been reported before.