Abstract Adult polycystic liver disease (APLD) is a rare disorder of liver parenchyma occasionally requiring surgical treatment. Its association with adult polycystic kidney disease has meant that as renal dialysis has become widely available there is an increased number of patients surviving with cystic liver changes. Although usually asymptomatic, patients with APLD may present with abdominal pain or swelling. Liver function is not usually compromised and computed tomography or abdominal ultrasonography are the most useful investigations. The complications of cyst rupture, infection, cholangiocarcinoma and compression of surrounding structures are discussed. Surgical treatment remains controversial, and the options of cyst puncture, fenestration with or without hepatic resection, and liver transplantation are reviewed.