Teratoma of the maxilla is a rare entity. Congenital intraoral teratoma occurs in 1:4000 births. It is a benign tumor, although malignancy has been described in adults. A 10-year-old male child with this condition is described in this report. This case illustrates a huge mass on the right side of the maxilla. The mass was excised under general anesthesia. Histopathologically, it consisted of all three layers of embryonic elements with predominantly fibrous tissue. Postoperative result was uneventful and no recurrence was detected after 2 years.
Keywords: Epignathus; maxilla; neonatal; teratoma.