Caroli’s disease is a rare inherited disorder. It has two types. The first is characterized by dilatation of the bile ducts, while the second form is associated with hepatic fibrosis and portal hypertension and is known as Caroli’s syndrome. Patients with these syndromes may have recurrent episodes of cholangitis and are also at risk for associated bacteremia, sepsis and cholangiocarcinoma. However, no specific treatment is available as yet. We present a case of an 18 year old male patient who presented with complaints of right sided abdominal pain and was diagnosed as Caroli’s disease to make a point that there is need to develop some guidelines on management of these cases to improve their quality of life and regress progression to malignancy as although rarely seen, this entity has potential to badly affect quality of life of the patients and turn malignant.