Recently, attention has been drawn to papillary neoplasm of the pancreatobiliary systems. In the pancreas, the disease entity of intraductal papillary mucinous neoplasm (IPMN-P) is widely recognized. In contrast, the pathological characteristics of biliary papillary tumors, such as biliary papilloma(tosis) and papillary cholangiocarcinoma, have not yet been well documented. In this study, we compared the pathological features and post-operative prognosis among biliary papillary tumors (10 cases of biliary papilloma(tosis) and 22 cases of papillary cholangiocarcinoma), conventional non-papillary cholangiocarcinoma (15 cases), and IPMN-P (31 cases). Macroscopically, all biliary papillary tumors were characterized by the prominent intraductal papillary proliferation, and macroscopic mucin-hypersecretion was seen in 9 of 32 cases (28%). Histologically, biliary papillary tumors consisted of three types of tumor cells (pancreaticobiliary, intestinal and gastric types), whereas only the pancreaticobiliary type was observed in non-papillary cholangiocarcinoma. Immunohistochemically, biliary papillary tumors were characterized by the common expression of MUC2, CDX2 and cytokeratin 20. In addition, biliary papillary tumors could be associated with two types of invasive lesions: tubular adenocarcinoma (9 cases) and mucinous carcinoma (5 cases). Patients with tubular adenocarcinoma had a poor prognosis compared to non-invasive papillary tumor or papillary tumor with mucinous carcinoma. These pathological characteristics and the survival status of biliary papillary tumors were different from those of non-papillary cholangiocarcinoma, and rather closely resembled those of IPMN-P. C ertain types of papillary tumors with different malignant potentials are known to occur in the intrahepatic and extrahepatic bile ducts. 1 Biliary papilloma (papilloma adenoma) is a rare benign neoplasm composed of papillary proliferation of the dysplastic biliary epithelium with delicate fibrovascular stalks. 1 Multiple recurring biliary papillomas are known as biliary papillomatosis. Some cases of biliary papillomatosis have multiple tumors at the same time, and others manifest asynchronous tumors at different sites of the biliary tract. 1,2 Biliary papilloma and papillomatosis are now thought to be pre-malignant lesions, and they have the potential to progress to invasive lesions via an adenomacarcinoma sequence. 3 Some cholangiocarcinomas show mainly papillary proliferation in the bile duct lumen, and those cases are designated as papillary cholangiocarcinoma (papillary-CC)