A Saudi family with three members afflicted with congenital hepatic fi
brosis (CHF) is described. CHF was associated with cystic dilatation o
f the intrahepatic bile ducts and autosomal dominant polycystic kidney
disease (ADPKD) in all of the three siblings. The proband's case repo
rt is presented and the detection of the other two children by family
screening is described. This is the first report of CHF in a Saudi fam
ily. Review of the disease is outlined.