Congenital hepatic fibrosis and adult-type autosomal dominant polycystic kidney disease in a child.

This reports a family with congenital hepatic fibrosis and adult polycystic kidney disease. Adult polycystic kidney disease was present in three generations. In addition, congenital hepatic fibrosis occurred in two members of the third generation. These conditions are generally held to have different modes of inheritance and the significance of their occurrence together is not clear.