A 45-year-old female was admitted to our hospital with sever abdominal fullness, and was diagnosed as polycystic liver disease with ultrasonography, abdominal CT and abdominal angiography. Almost all part of the right lobe of the liver was replaced with a lot of cysts. The liver obviously swelled up. We thought her symptom came from oppression of the digestive tract by the liver. Liver function is good. Right lobe resection, deroofing and fenestration were performed, and after operation the volume of the liver was reduced and the symptom got better. We report a case of polycystic liver disease improved with surgical therapy, adding some study with reference.