Introduction <p>Epignathus is a rare congenital teratoma, often associated with a high risk of upper airway obstruction. This case report describes a full-term neonate born with no significant prenatal findings.</p> Case report <p>At birth, the infant exhibited severe respiratory distress related to a tumor originating from the right hard palate, obstructing the airway, along with a cleft palate. The newborn was subsequently hospitalized for eight days, requiring positive pressure ventilation and surgical resection of the tumor, which was performed on day five followed by favorable clinical progress. Diagnostic imaging confirmed a teratoma with dental structures inside, and elevated levels of alpha-fetoprotein detected in blood tumor markers supported the diagnosis. Histopathological analysis confirmed the tumor as a teratoma.</p> Discussion and conclusions <p>Despite the high neonatal mortality associated with epignathus due to airway obstruction, the aim of this article is highlight the importance of early multidisciplinary intervention, as well as carry out an updated review of the disease.</p>