Congenital hepatic fibrosis is an uncommon fibrocystic disorder affecting the intrahepatic bile ducts. It has autosomal recessive inheritance. The main consequence of this condition is portal hypertension and it is often misdiagnosed as cirrhosis. Patients with congenital hepatic fibrosis usually present during childhood or early adolescence with oesophageal variceal bleeding. Portosystemic shunt surgery is the treatment of choice for these patients as the risk of postoperative hepatic encephalopathy is low. We report a patient with congenital hepatic fibrosis who presented with oesophageal variceal bleeding at the age of 16 years, initially misdiagnosed as having cryptogenic liver cirrhosis. The patient experienced two further episodes of oesophageal variceal bleeding in subsequent years. She eventually underwent portosystemic shunt surgery. One year after the operation, the shunt remained patent on Doppler ultrasonography, and there had been no further episodes of variceal bleeding post-surgery.