A 48-year-old female nurse presents with abdominal pain and hepatomegaly. There is a family history of polycystic kidney disease. The pain is severe and using a visual analogue scale (VAS 0-10) for abdominal pain the score is 9-10, and with no association to food intake. She is unable to work full time as a nurse and has lost 3-4 kg of body weight. The pain has been present for more than 9 months with worsening during the last 3-4 months and the patient has also impression of progression of hepatomegaly. The serum liver tests are normal apart from slightly elevated alkaline phosphatase and bilirubin. At physical examination there is marked hepatomegaly. Ultrasound and later a CT-scan demonstrate multiple small-medium sized cysts in both liver lobes with sparse normal liver tissue. In addition, there is ascites and additional signs of portal hypertension with collaterals. There are also several cysts detected in both kidneys. Kidney function is normal.