Sarcomatoid differentiation is a rare and aggressive histologic subtype with poor prognosis, seen in several malignancies. In sarcomatoid renal cell carcinoma (RCC), the degree of sarcomatoid differentiation and the stage at presentation determines the prognosis. Despite resection, chemotherapy and targeted therapy response is modest, with relapse usually occurring within a few months. We present a case of a gentleman with sarcomatoid RCC managed with pembrolizumab, who has had no evidence of recurrence for over 4 years since the last dose of immunotherapy. RCCs with sarcomatoid differentiation have a high presence of programmed cell death protein 1 and programmed cell death ligand 1 in T cells and tumor cells, respectively, making immunotherapy an attractive option in this setting. Clinical trials are ongoing to further define the benefit of immunotherapy in sarcomatoid RCC.