Introduction: Chromophobe renal cell carcinoma is universally accepted as a distinct subtype of renal cell carcinoma. There are conflicting reports on prognosis, and few data on response to treatment exist. Currently, we do not have any effective treatment for the metastatic disease apart from surgical procedures. We report unexpectedly high efficacy of temsirolimus in a patient, with metastatic chromophobe renal cell carcinoma of right kidney. Case Report: We present the case of a 50-year-old female patient with advanced chromophobe renal cell carcinoma of right kidney, relapsing after radical nephrectomy; she was diagnosed of mRCC (metastatic renal cell carcinoma) with multiple lung metastasis. Treatment with temsirolimus resulted in a stable disease for 48 cycles. Conclusion: Mammalian target of rapamycin inhibitors like temsirolimus have been an option in the management of such cases. The case presented here impresses that temsirolimus could be a viable option for patients with metastatic chromophobe renal cell carcinoma.