Extraosseous Ewing sarcoma with genitourinary tract involvement is rare. We present a case of primary Ewing sarcoma of the testis with review of the literature. While primary Ewing sarcoma of the testis is unusual, it is important to appropriately diagnose. This case emphasizes the benefits of prompt and appropriate evaluation, pathologic work-up, and treatment of testicular tumors. [ABSTRACT FROM AUTHOR]