Uterine rhabdomyosarcoma is exceedingly rare. The treatment strategy has evolved from aggressive local control with upfront surgery followed by radiation to a more conservative approach with chemotherapy followed by additional treatment pending response, which is outlined in a recent consensus statement from the International Soft-Tissue Sarcoma Consortium. We present a case of a 2-year-old with intermediate risk uterine rhabdomyosarcoma. She was treated with a multimodal regimen including chemotherapy, radiation, and surgery with abdominal hysterectomy, bilateral salpingectomy, oophoropexy, partial proximal vaginectomy, and right extravesical nonrefluxing ureteral reimplant to achieve a complete resection.