Polycystic liver disease is a rare genetic condition that is characterized by progressive development of fluid-filled biliary epithelial cysts throughout all segments of the liver. A normal liver has a smooth, uniform appearance. A polycystic liver can look like a cluster of very large grapes, of various sizes scattered throughout the liver. Cysts also can grow independently in different parts of the liver. The cysts, if they get too numerous or large, may cause abdominal discomfort and distention, early satiety and supine dyspnoea. Fortunately, liver cysts rarely cause any symptoms or liver problems, though the cysts may cause pain if they press against other organs. A minority of people will need surgery to reduce the size of the liver. Fenestration is an acceptable procedure for patients with a dominant cyst pattern where liver size can be reduced after the cysts collapse. A combination of resection-fenestration is suitable for those with a diffuse cyst pattern where grossly affected segments are resected in combination with fenestration to allow for reduction in liver size.