Nine cases of endodermal sinus tumor of the vagina from children 6 months to 2 years of age are reported. The two patients treated before the advent of modern chemotherapy died 6 to 13 months postoperatively despite attempted complete surgical resection of the tumor in one of the cases. One patient who was treated initially by hysterectomy and vaginectomy had lung metastases 6 months postoperatively and died despite chemotherapy. Six patients who were treated by a surgical procedure and received vincristine, actinomycin D, and cyclophosphamide, with radiation therapy in two cases, were alive and free of disease from 2 to 9 years postoperatively.