This article reviews the current available information on Ewing's sarcoma of the urogenital tract, focusing on the presentation, diagnosis, and therapeutic management of this uncommon entity. Because of the rapid local growth of these tumors, an immediate decision for treatment initiation is mandatory. The classical presentation depends mainly on the site of involvement of these tumors, involving a palpable mass, hematuria, and pain. No specific diagnostic imaging is available to date, and the only diagnostic method remains histological examination in combination with immunohistochemistry. Treatment involves primary surgical resection of all tumor tissues followed by adjuvant multi-agent chemotherapy. Overall survival is poor, although a 13-year survival has been reached in one case.