Renal cell carcinoma (RCC) consists of a heterogeneous group of tumors with distinct genetic and metabolic defects, as well as histopathologic and clinical features. RCC has an inclination for metastatic spread to uncommon sites, with an aggressive behaviour. Here, we share an unusual case of sarcomatoid RCC (sRCC) with lung metastasis, in a patient who underwent left-sided radical nephrectomy nearly 15 years ago. We used an emerging treatment option, with a positive outcome in the case.