Biliary papillomatosis is rare. Before 1993, there were only 21 cases reported in the English literature. This article reports our experience in managing this disease and reviews experiences from recent English literature. A retrospective review of patients with biliary papillomatosis was conducted in our institution. A search of the English literature between 1993 and 2002 was performed in the MEDLINE database using "biliary papillomatosis" as the keyword. Seven patients with biliary papillomatosis were treated in our hospital. Another 50 cases were reported in the recent literature. This amounted to a total of 78 cases so far reported. The male to female ratio was approximately 2 : 1. The mean age at presentation was 63 years. Most patients presented typically with cholangitis. Malignant change was present in 33 patients (42%). Concomitant intrahepatic and extrahepatic disease was noticed in 33 patients (42%). The overall resection rate was 55%. The mean survival was 28 months following resection, irrespective of histological type. Patients without tumor resection had a poorer survival of less than 11 months. Biliary papillomatosis presents typically with biliary obstruction and it occurs most often in elderly males. Resection is recommended because of the considerable malignancy rate, diffuse pattern of disease, and better survival following curative surgery. Malignant histology is not always associated with a poorer prognosis provided that radical resection can be achieved.