Congenital hepatic fibrosis (CHF) is characterized by periportal fibrosis with irregulary shaped proliferating bile ducts and is recognized as a member of the fibrocystic disease family. It is such a rare disorder that only about 200 patients with CHF has been reported. Affected children or adults often show portal hypertension and related complications, which may lead to bleeding from gastroesophageal varices. The diagnosis of CHF is usually confirmed by its typical histological features. The patients with portal hypertension due to CHF are ideal candidates for portosystemic shunt because most of them have good liver function. Recently, we experienced a case of CHF without hepatic and renal lesions presenting with hematemesis. Endoscopic variceal ligation was performed. We report this case with a review of relevant literatures.