Congenital teratomas of the oral cavity commonly present as tissue masses projecting from the mouth. The important prognostic determinants are: the age of presentation, technical problems during surgical removal of the mass, associated anomalies, and the nature of the composing tissues. This study reports one case of epignathus (an extremely rare oropharyngeal teratoma) that was diagnosed by ultrasonography at 19 weeks of gestation and reviews the relevant literature.