Sarcoma botryoides, a variant of embryonal rhabdomyosarcoma, is the most common neoplasm of the lower genital tract in girls under 16 years of age. This neoplasm more frequently has a vaginal location in patients younger than 4 years, whereas a cervical location has its peak incidence in the second decade. Although the recommended therapy has been radical surgery alone or combined with other modalities, the use of neoadjuvant chemotherapy has changed the surgical approach to one employing a less radical procedure such as a simple hysterectomy or local excision. We present the case of a 12-year-old girl diagnosed with an embryonal rhabdomyosarcoma of the endocervix who received vincristine-Adriamycin-cyclophosphamide combination chemotherapy followed by a simple hysterectomy. The patient exhibits no evidence of disease 8 years after the initial diagnosis.