Ewing's sarcoma/Primitive Neuro-Ectodermic Tumor of the kidney accounts for less than 1% of all kidney tumors. We present the case of a 37-year-old male with abdominal pain secondary to a right renal tumor of 7 × 7 × 5 cm. A robotic right radical nephrectomy was performed. In the histopathological and immunohistochemistry was reported NKX2.2(+), CD99(+), synaptophysin(+), and FLI-1(+). Extraosseous Ewing's Sarcoma was diagnosed. The patient was treated with: Doxorubicin, Vincristine, Mesna, and Isophosphamide.