In a 66-year-old woman with pruritus, jaundice, dark-brown urine and light-colored faeces obstructive jaundice was diagnosed. Despite extensive investigations, it was not possible to clearly distinguish if varicosis or cholangiocarcinoma was the cause of the obstruction. During laparotomy the right lobe of the liver was seen to be greatly underdeveloped. The portal system showed a varicose deformation with compression of the bile ducts and portal hypertension. The right lobe of the liver was removed and the portal hypertension was treated by creating a shunt between the hepatic portal vein and the right ovarian vein. The jaundice disappeared and the patient recovered. Histological investigation showed atrophy, secondary biliary fibrosis, cirrhosis and a biliary cystadenoma. There were no signs of malignancy. The varicose deformation can be considered to be a result of the portal hypertension caused by fibrosis and cirrhosis with possibly a history of thrombosis and insufficient recanalization.