Caroli's disease: diagnosed by ERCP and ultrasonography.

A patient with Caroli's syndrome is reported; diagnosis was made by ERCP and ultrasonography. Caroli's disease occurred in its "pure" form in this patient, i.e. dilatation was present only in the intrahepatic biliary system while the extrahepatic bile ducts were normal. Pathogenesis and differential diagnosis are surveyed on the basis of available data. Further, the possibilities of treatment and prognosis are dealt with.