Wilson's disease is a rare, autosomal-recessive inherited disorder of copper metabolism resulting in accumulation of copper in liver. The form of liver disease varies, depending on the severity of the disease at the time of diagnosis and pathological findings include fatty changes, acute hepatitis, chronic active hepatitis, cirrhosis and occasionally fulminant hepatic necrosis. Liver imaging findings reflect a wide range of physiopathological processes of the disease and also demonstrate the associated findings of cirrhosis in cases with advanced disease.