Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys.

A case report is presented of a child who died in the neonatal period and who had the sonographic findings of multiple, tubular, fluid-filled spaces in the liver characteristic of Caroli's disease. There was an echodense renal cortex and pyramids. Necropsy findings were consistent with Caroli's disease but the liver also showed evidence of congenital hepatic fibrosis and there was infantile type polycystic disease of the kidneys.