Congenital hepatic fibrosis(CHF) is a rare development abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all. cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The patient also had cystic dilation of subarchnoid space in the basal ciatern and posterrior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. Ha had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures. (Korean J Hepatol 2001;7 :485 - 490)
