The incidence of malignant change of ovarian mature teratoma is 1~2%. The majority is squamous cell cancer, the others was adenocarcinoma. Neuroepithelial tissue was frequently detected in mature cystic teratoma, but their malignant change was extremely rare. Only, two cases of neuroblastoma of ovarian teratoma were reported in the world. We report one case of neuroblastoma arising in ovarian mature teratoma with a brief review. Our case is the third reported one in the world.