An epignathus is an oropharyngeal teratoma composed of cells from ectodermal, mesodermal, and endodermal layers. Epignathi that arise from the palate or pharynx and protrude from the mouth result in life-threatening airway obstruction and usually cause asphyxiation shortly after birth. In our reported case, an antenatal ultrasound diagnosis allowed for preparation of an ex utero intrapartum treatment (EXIT) procedure. A tracheostomy was performed at birth with maternal-fetal blood flow still intact through the umbilical cord. Debulking of the large extraoral portion of the tumor, followed by complete intraoral resection, was performed. Masseteric function and swallowing slowly improved over several weeks. Cleft palate repair is planned at 10 months of age. In the following report, the histology, classification, and pathogenesis of these "parasitic" tumors are reviewed.