Tumors of neuroepithelial origin are extremely rare in teratoma and tend to be derived from glial or primitive neuroectodermal cells. We describe a case of 2- month-old baby girl with an oligodendroglioma arising in an immature teratoma of the sacrococcygeal region. Histologically, the tumor was identical in appearance to low grade oligodendroglioma within the adult brain. Because immature teratoma was grade II, the patient received adjuvant chemotherapy. The patient died of progression of the intra-abdominal tumor 6 months after surgical excision. The authors believe this to be the first presentation in the world literature.