Polycystic liver disease (PLD) is commonly associated with polycystic kidney disease, but is unaccompanied by renal cysts in 10% of cases. Symptomatic PLD is mainly limited to adults and rarely, if ever, presents in childhood. There is currently no effective medical therapy for PLD. Most patients who have PLD require no medical or surgical intervention. Patients with PLD rarely, if ever, experience biochemical hepatic deterioration, and classic symptoms of hepatic failure such as ascites, variceal hemorrhage, or encephalopathy are unusual. [1][2][3] Although there is a typical lowering of the quality of life in these patients, to date, quality of life (although not well studied) has not been associated with decreased survival. Serious, life-threatening complications related to multiple hepatic cysts may occur, including cyst infection, hepatic vein outflow obstruction, portal hypertension, refractory ascites after cyst fenestration, cyst carcinoma, cholangiocarcinoma, and most importantly, pain and/or chronic wasting with malnutrition. [2][3][4] Symptomatic PLD only occurs in patients with massive hepatic cystic disease in which the total cyst: parenchyma ratio is ΟΎ1. 5 Patients with PLD on hemodialysis are at greatest risk of life-threatening complications due to hepatic cysts complicated by infection, hemorrhage, and carcinoma. In one center, 10% of the mortality in patients with PLD on hemodialysis was attributed to these complications. However, this observation has not been confirmed by other centers. Nontransplant interventions that can relieve symptoms of massive hepatic cystic disease include percutaneous cyst puncture and sclerotherapy, laparoscopic cyst decompression, open laparotomy with fenestration, and liver resection. 3,[6][7][8][9][10] These interventions can provide temporary or prolonged relief of symptoms but may be associated with significant morbidity and