Peribiliary cysts, known as cystic dilatation, of the extramural peribiliary glands of the bile duct are rare, and are usually detectable under conditions of pre-existing hepatobiliary diseases such as liver cirrhosis. Preoperative diagnosis is often difficult, because they are usually asymptomatic. Distinction of peribiliary cysts from premalignant or malignant cystic lesions is mandatory. Herein, we report a case of peribiliary cyst, which was preoperatively assumed as unilobar Caroli's diseases in healthy young patients and briefly discuss the management of the condition.