Congenital hepatic fibrosis (CHF) was detected in a 10-year-old boy with hepatosplenomegaly and leukopenia and in a 45-year-old man with hepatomegaly. Several years after these original findings, both patients developed bleeding that was attributed to esophageal varices and were treated by elective portasystemic shunts. In both, the preshunt liver function tests were essentially normal and the liver scans revealed homogeneous uptake. The gross aspect of the livers at laparotomy was indistinguishable from that of cirrhosis, and the liver wedge biopsies showed generalized portal and interlobular fibrosis and bile-duct hyperplasia but no evidence of previous liver-cell necrosis. Hepatic vein catheterization and portal venography in the adult patient indicated intrahepatic presinusoidal block. Neither patient had jaundice, ascites, or polycystic kidneys. Both cases appear sporadic.