Congenital hepatic fibrosis (CHF) is an autosomal recessive disease characterized
histologically by a variable degree of periportal fibrosis and irregularly shaped proliferating
bile ducts. It is one of the fibropolycystic diseases.
CHF manifests itself clinically with hepatosplenomegaly, portal hypertension and its related
complications. Hepatocellular function is usually well preserved. Other clinical features of
CHF include recurrent cholangitis, especially in patients with CHF and Caroli’s syndrome (CS).
Patients usually develop nonspecific symptoms, making the diagnosis of this disorder extremely
difficult. Most cases are diagnosed during adolescence and early adulthood.
Histopathologic findings on liver biopsy are the gold standard for diagnosis of CHF.