Biliary papillomatosis is a rare entity, characterized by single or multiple lesions arising from the biliary epithelium, leading to relapsing attacks of obstructive jaundice and cholangitis. Usually considered a benign disease, progression to malignancy has though been reported. In this paper the case of a 72 year old patient, with a single papilloma of the left hepatic duct treated by left hepatectomy, is discussed. The main histological, clinical and therapeutic aspects of this unusual pathology are then examined, with a complete review of the Literature.