Central diabetes insipidus associated with Caroli syndrome

Caroli disease is a rare congenital malformation characterized by cystic dilatations of large bile ducts. There are two forms of Caroli disese. Simple form entails the bile duct dilatation or ectasia. Complex form is copresense of hepatic fibrosis and portal hypertension. Here is presented a case of central diabetes insipidus (CDI) associated with Caroli disease.