Nowadays liver and biliary tract surgery are the most commonly performed procedures worldwide; refinement in surgical techniques, anesthetic care and liver transplantation have revolutionized the treatment of liver diseases that were previously incurable. The autosomal dominant polycystic liver disease is a rare condition that may be associated with polycystic kidney disease or can present alone; the symptoms of this condition occur in the advanced stages of the disease. Despite the multiple modalities of treatment available, surgery with hepatectomy and fenestration has shown better results in patients with early satiety and massive hepatomegaly. A literature review was carried out and a number of cases dealt with this disease at the Metropolitan Hospital in Quito, Ecuador, are presented.