Teratomas are rare germ cell tumors that comprise tilde; 1 percnt; of orbital tumors in childhood. Review of the world literature revealed only 51 well-documented patients with true congenital orbital teratomas. We present a newborn girl with a massive orbital teratoma that caused significant orbital enlargement with inferior and lateral displacement of the zygoma and a thinning of the orbital roof. The ipsilateral maxilla and palate were depressed inferiorly. No bony invasion was seen despite its massive size. A craniofacial approach was used to safely and completely extirpate this tumor. Histological sections demonstrated derivatives of all three germ cell layers. Recommendations for definitive treatment and a review of the literature are presented.
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