Collecting duct carcinoma (CDC) is a rare, highly aggressive malignant neoplasm that arises from the collecting duct epithelium of the kidney. CDC was reported to coexist with renal cell and transitional cell carcinomas. We report a rare case of CDC associated with oncocytoma, confirmed by the characteristic histological appearance and immunohistochemistry. We also review the epidemiological, histological and immunohistochemical criteria for diagnosis, in addition to the genetic and cytogenetic aberrations reported in the literature. Identification and reporting CDC is important for the establishment of treatment strategies and monitoring prognosis.