Endodermal sinus tumors are rare and highly malignant germ cell tumors in both gonadal and extragonadal tissues. Primary vaginal endodermal sinus tomors(such as present case) are predominatly tumors of infants, and carry a dismal prognosis. The recent occurrence of such a neoplasm, in an 18-month-old child treated postoperatively with multiple courses of combined chemotherapy followed by apparent improvement in the level of alpha-fetoprotein, prompted us to review the lassical and current world literature on these tuumors. This appears to be the first case reported in Korea.