Introduction: Collecting duct carcinoma (CDC) is a rare, aggressive form of renal carcinoma that presents at an advanced stage and has a poor prognosis. Little is known concerning the optimal management of CDC. We present the results of a systematic review addressing the management of CDC and the McMaster University CDC series. Methods: The MEDLINE, Cochrane Library, and EMBASE databases and conference proceedings were searched to identify studies relating to the management of CDC. Included studies reported on a minimum of 10 subjects receiving a single intervention. Series in which an evaluation of therapeutic effectiveness was not possible were excluded. The McMaster University (Hamilton, Ontario) series of 6 cases of CDC were retrospectively reviewed. Results: We identified 3 studies relevant to the management of CDC that included a total of 72 patients. A gemcitabine–cisplatin or –carboplatin regimen resulted in a 26% objective response rate in 23 patients with metastatic CDC. Two additional studies indicated that 49 patients treated with immunotherapy achieved no response. In the McMaster series, cytoreductive nephrectomy was performed in 4 of 6 patients. In 2 patients, MVAC therapy (methotrexate–vinblastine–doxorubicin–cisplatin) achieved no response. No significant therapeutic complications occurred, but survival was poor (median: 11 months; range: 10–33 months). Conclusions: Our review and clinical experience suggest that the current standard of care for metastatic CDC is a gemcitabine–cisplatin regimen.