Adenocarcinoma accounts for a small percentage of neoplasms arising within the renal pelvis. We describe a mucinous adenocarcinoma of the renal pelvis that occurred in a 57-year-old woman. Investigation of the recent literature reveals an additional 12 cases of adenocarcinoma of the renal pelvis reported since 1980. These 13 cases are summarized in detail, for a total of 59 cases of adenocarcinoma of the renal pelvis documented in the English-language literature. These tumors can be subdivided into three major histologic types: tubulovillous, mucinous, and papillary non-intestinal. The tubulovillous and mucinous tumors are morphologically similar to intestinal tumors accounting for 71.5% and 21.5% of cases, respectively. They are believed to arise in foci of intestinal metaplasia. Only three cases (7%) were of the nonintestinal, nonmucinous, papillary subtype. These rare tumors are notable for their morphologic similarity to Bellini or collecting duct carcinoma, but a specific morphologic precursor has not been identified. Of the three subtypes, tumors of tubulovillous morphology confer the worst prognosis with 70% of patients dying within 5 years. Thirty-three percent of mucinous tumors and none of the papillary nonintestinal tumors were fatal.