INTRODUCTION: The PLD, included in the group of malformative lesions of ductal plate, is characterized by progressive development of multiple parenchymal cysts. Different surgical treatments have been proposed for symptomatic patients with PLD: percutaneus aspiration, fenestration, hepatic resection and liver transplantation. The aim of this report is to outline the treatment of polycystic liver disease with laparoscopic fenestration. MATERIALS AND METHODS: Of thirteen patients with PLD, in all cases associated with polycystic kidney disease, 7 patients underwent to laparoscopic treatment of cysts fenestration, and 6, asymptomatic and not amenable to surgical treatment, underwent to clinical follow-up. Of the 7 patients, 5 have symptoms related to “effect mass” 1-2-3 of hepatic cysts while in the 2 asymptomatics the fenestration of the cysts was performed during the procedure of laparoscopic cholecystectomy for cholelithiasis. DISCUSSION: The best indication for laparoscopic fenestration are those cases of PLD characterized by a relatively limited number of large cysts, preferably situated in the anterior segments of the liver and in the left lobe; in this patients, laparoscopic fenestration reduces significantly the volume of the liver and relieves symptoms. The laparoscopic fenestration of hepatic cyst, in carefully selected patients, is an effective technique in terms of morbidity, mortality, conversion rate and recurrence rates; while in patients with cyst diffuse in liver parenchyma is indicated the hepatic resection or liver transplantation. The optimal surgical approach is still evolving, the type of approach is related to extent and distribution of the cysts, and vascular anatomy of normal segment of the liver.