Teratoma of the tongue is a rare tumor of the oral cavity with only six reported cases in the literature. Teratomas are composed of ectoderm, mesoderm and endoderm with differentiation to identifiable tissues and organs. Embryologically, they are thought to occur as a result of displacement of cells from normal tissue during fetal life. In the tongue, the teratoma may result from misplaced cells from the tuberculum impar. Differential diagnosis of tongue lesions includes thyroglossal duct cyst, lingual thyroid, lymphangioma, hemangioma, dermoid cyst, granular cell myoblastoma and heterotopic gastric mucosal cyst. Computerized tomography is useful in differential diagnosis and defining extent of disease. Surgical excision with laser is curative and recurrence is rare.