Background: Epignathus is a rare kind of teratoma. Its estimated incidence is one in 35,000–200,000 live births, and it may cause high mortality by causing airway obstruction in the neonatal period. It is generally diagnosed in the prenatal period. Prenatal diagnosis and follow-up are important for multidisciplinary preparation during delivery. Ultrasound and magnetic resonance imaging are used in its diagnosis and location. If appropriate, the extra-uterine intrapartum treatment procedure to excise the mass or open the airway and future palate repair are current treatment options. Case Presentation: In this report, we will present an epignathus originating from hard palate and protruding from the oral cavity. It was treated by EXIT procedure successfully. Conclusion: Congenital epignathus is a rare anomaly that can threaten the life of the newborn at birth. Where appropriate, the EXIT procedure provides successful treatment.