Sarcomatoid renal cell carcinoma represents high-grade transformation of different subtypes of renal cell carcinoma and is associated with a dismal prognosis and high resistance to chemotherapy. We report on the course of disease of a 63 years old patient undergoing a nearly complete remission of multiple intracranial and spinal metastatic lesions of a sarcomatoid renal cell carcinoma by a combined chemotherapy with temozolomide and pegylated liposomal doxorubicin.