Isolated hepatic cysts are commonly detected incidentally on abdominal ultrasound or CT ( 1 ). These cysts are usually asymptomatic and have no clinical significance. The rare congenital polycystic liver is commonly associated with polycystic disease of the kidneys and other organs. It causes progressive nodular hepatomegaly (sometimes massive) in adults. Nevertheless, hepatocellular function is remarkably well preserved, and portal hypertension rarely develops. Occasionally, very large cysts cause pain or symptoms from compression of other organs. In these instances, intervention such as cyst marsupialization or drainage can be considered; however, cysts often recur. Thus, rarely, significant symptoms or quality-of-life issues warrant consideration of liver transplantation ( 1 ).