Polycystic liver disease is an autosomal dominant disorder, rare and benign in the majority of cases [1]. It is frequently associated with polycystic kidney disease, but can also be isolated in rare cases. Liver cysts result from abnormal growth of the biliary epithelium (cholangiocytes) or dilation of the peribiliary glands, due to the persistence of embryonic biliary structures. Isolated polycystic liver disease is generally asymptomatic, women are more affected than men and also have a greater number of cysts [2]. We report the case of a 40-year-old asymptomatic man with isolated polycystic liver disease Gigot III discovered during a health check-up. The clinical examination showed hepatomegaly without pain, of firm consistency, smooth surface, sharp lower edge with a hepatic arrow at 17cm. The kidneys are devoid of cysts, the liver function as well as the renal function are normal.