Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease. Cases have been reported from all over the world but the exact incidence of the disease is not known. The diagnosis sometimes is difficult to establish and one of the main diagnostic method is histological evaluation. The management and prognosis of CHF is dependent on alimentary bleeding secondary to portal hypertension. In late childhood abdominal pain, cholangitis and features of hypersplenism complicate the problem. Herein we present the case report of patient with CHF. Our choice of treatment was living donor liver transplantation. This procedure is a very difficult but only life – saving chance for patients with CHF.
liver fibrosis, living donor liver transplantation, congenital hepatic fibrosis, chronic liver failure