The endodermal sinus tumor of ovary is a rare, malignant germ cell tumor. It principally affects children and young adults. Earlier reports before combination chemothery described it was fatal and the prognosis was poor. The advances in treatment of endodermal sinus tumor have been achieved with the development of combination chemotherapy with monitoring tumor marker, serum AFP. The authors experienced a case of endodermal sinus tumor of ovary which showed complete remission following primary conservative surgery and three courses of combination chemotherapy(BEP) with normal full-term spontaneous delivery. With the case report, a brief review was made.