Abstract
An unusual case of intrahepatic biliary ectasia and congenital hepatic fibrosis confined to one segment of the liver is described in a young woman who died from a ruptured splenic artery aneurysm in the third trimester of pregnancy. The possible mechanisms for production of the severe portal hypertension in this patient and the association of an aneurysm in the splenic artery are discussed. The patients karyotype was 45, XX, -D, -D, +t (13q 15q).