Polycystic liver disease (PLD) is a genetic disorder [1] that is di- agnosed by presence of multiple hepatic cysts. Clinical manifesta- tions of PLD are related to the size of hepatic cysts and associated complications. In the early stage, PLD rarely causes any symptoms. However, patients with advanced stage PLD have a variety of clin- ical manifestations and complications due to severe hepatomegaly. They include abdominal pain, abdominal distension, dyspepsia, as- cites and many others that may result from compression of ad- jacent tissue or organ failure [ 2 , 3 ]. Surgical treatment of PLD in- cludes: aspiration with or without sclerotherapy, cyst fenestration, hepatectomy and liver transplantation. Despite some researchers have concluded that cyst fenestration or hepatectomy is effective in treatment of PLD [4–6] , the only curative surgical option in se- vere patients is liver transplantation [ 7 , 8 ]. All other types of surg- eries present a great challenge to a surgeon, having little or no ef- fect when cysts are diffuse, or even contraindicated due to possi- bility of life-threatening complications arising during or after the procedure. Herein, we report a case of 51-year-old male diagnosed with massive PLD complicated by abdominal adhesion, liver cirrho- sis, splanchnic varices and ascites that successfully underwent liver transplantation.