The sarcomatoid variant of renal-cell carcinoma (SRCC), a clinically aggressive subtype of renal parenchymal tumors, is typically resistant to systemic treatments and carries a poor prognosis. The authors report a case of a 57-year-old male with advanced SRCC who had a durable complete response after MAID (mesna, adriamycin, ifosfamide and dacarbazine) chemotherapy, and remains free of disease four years after completing treatment. To the authors' knowledge, this is the first report of a remission from MAID chemotherapy in SRCC. A review of published literature revealed occasional responses after systemic chemotherapy. Notably, all responses were seen with doxorubicin containing regimens, suggesting that doxorubicin is a critical component in chemotherapy regimens for SRCC.