Sarcomatoid renal cell carcinoma (SRCC) is a heterogeneous disease with generally unreliable response to various therapies in clinical studies. We illustrate a case report in which a woman with metastatic SRCC had a complete and durable response to adriamycin and ifosfamide chemotherapy. We find this to be incongruous with expectations from some recently published data. It underscores the fact that the biology of SRCC needs to be studied in more detail for further subcataloging of the disease into diverse prognostic categories.