Caroli's disease in children: Is it commonly misdiagnosed?

Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding. Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.