Endodermal sinus tumor of the vagina, a rare pediatric malignancy, was managed with primary chemotherapy (vincristine, dactinomycin, cyclophosphamide [VAC]) followed by local excision without radical surgery or pelvic irradiation. Treatment-related complications were acceptable, and there is no evidence of disease 30 months after discontinuing all therapy (50 months after diagnosis). As a treatment option, primary chemotherapy is viewed as a highly attractive alternative modality, preserving potential reproductive and sexual function in long-term survivors, and appears to be the treatment of choice.