Congenital Hepatic fibrosis is an uncommon disease, which is autosomal recessive. Two forms of the disease are distinguished: a rare one becoming manifest in the neonatal period with signs of progressive renal failure secondary to polycystic kidneys, in such cases the liver fibrosis is usually asymptomatic, and the diagnosis is therefore often first established post mortem. In the other more usual form the patients present during infancy with bleeding from rupture of esophageal varices caused by portal hypertension. One case is reported, and the history of his two brothers are referred to. In our case a portacaval anastomosis was performed and the patient is asymptomatic with no signs of encephalopathy. His two brothers died, one of bleeding from esophageal varices, the other of hemolytic anemia. In both cases the diagnosis was first made post mortem.