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Gynecologic tumors in children are rare and represent < 5% of all pediatric neoplasms. Rhabdomyosarcoma is a soft tissue tumor arising from primitive mesenchymal cells which retain their capacity for striated skeletal muscle differentiation. The most common site is the head and neck region, followed by the genitourinary tract. We report a case of a 15 year-old girl who presented with a mass descending from the vagina. Histopathology and IHC studies confirmed Embryonal Botryoid Rhabdomyosarcoma. She underwent a type 2 radical hysterectomy followed by six cycles of the VAC regime (Vincristine, Dactinomycin and Cyclophosphamide). This case is reported due to the rarity of presentation and to document the treatment modalities of this condition. A systematic review of the literature was also done. The review showed that larger tumors and metastatic disease had poorer outcomes.