Polycystic Kidney of Autosomal Dominant Inheritance, Polycystic Liver and Congenital Hepatic Fibrosis in a Single Kindred

A family with clear evidence of concomitant congenital hepatic fibrosis, polycystic liver and adult-type polycystic kidney is reported. The autosomal dominant inheritance of polycystic kidney in association with either liver cyst or hepatic fibrosis in this family suggests that various combinations of hepatic and renal fibrocystic lesions are possible to occur. Additionally, in the proband a very rare association of congenital hepatic fibrosis and cerebral aneurysm was present.