Teratomas are rare germ cell tumors that comprise similar to 1% of orb
ital tumors in childhood. Review of the world literature revealed only
51 well-documented patients with true congenital orbital teratomas. W
e present a newborn girl with a massive orbital teratoma that caused s
ignificant orbital enlargement with inferior and lateral displacement
of the zygoma and a thinning of the orbital roof. The ipsilateral maxi
lla and palate were depressed inferiorly. No bony invasion was seen de
spite its massive size. A craniofacial approach was used to safely and
completely extirpate this tumor. Histological sections demonstrated d
erivatives of all three germ cell layers. Recommendations for definiti
ve treatment and a review of the literature are presented.