Ewing sarcoma or primitive neuroectodermal tumor (ES/PNET) is an aggressive type of sarcoma that is rarely observed in the kidney. The diagnosis of primary renal EWS can be difficult; it mostly presents with non specific symptoms. The final diagnosis is based on combination of immunohistochemistry and fluorescence in situ hybridization (FISH). We review here in a case of Ewing Sarcoma of the kidney, and we review briefly the existing literature data.