A patient with adult polycystic liver and kidney disease presented with haematemesis and melaena and was found to have raised serum creatinine, aspartate transaminase, and alkaline phosphatase values; hypoalbuminaemia; and a prolonged prothrombin ratio. She also had oesophageal varices. With haemodialysis her aspartate transaminase activity fell to normal but she remained hypoalbuminaemic with a prolonged prothrombin ratio. She died after three weeks. Although hepatic cysts do occur in adult polycystic kidney disease, they have been thought not to cause major liver disease. The hepatic cysts in this patient, however, did appear to be associated with portal hypertension and impaired hepatocellular function.