45 year old man presented with renal insufficiency. Liver function tests were normal. Abdominal palpation revealed an enlarged liver.
The liver is irregularly enlarged by the presence of innumerable round, thin-walled cysts of variable sizes, some of which are subcapsular. The cysts contain clear fluid and have smooth inner surfaces. One large cyst has blood-stained contents.
The intrahepatic bile ducts are not dilated. No calculus or cirrhosis is seen.