Papillary tumor of the bile duct is characterized by the presence of an intraductal tumor with a papillary surface comprising innumerable frondlike infoldings of proliferated columnar epithelial cells surrounding slender fibrovascular stalks. There may be multiple tumors along the bile ducts (papillomatosis or papillary carcinomatosis), which are dilated due to obstruction by a tumor per se, by sloughed tumor debris, or by excessive mucin. Radiologically, the biliary tree is diffusely dilated, either in a lobar or segmental fashion, or aneurysmally, depending on the location of the tumor, the debris, and the amount of mucin production. A tumor can be depicted by imaging as an intraductal mass with a thickened and irregular bile duct wall. Sloughed tumor debris and mucin plugs should be differentiated from bile duct stones. Cystically or aneurysmally, dilated bile ducts in mucin-hypersecreting variants (intraductal papillary mucinous tumors) should be differentiated from cystadenoma, cystadenocarcinoma and liver abscess.