Abstract
Epignathus is a rare congenital oropharyngeal teratoma that arises from the oropharynx, especially the sphenoid, palatine, and ethmoid bones. Teratomas are benign tumors containing cells from ectodermal, mesodermal, and endodermal layers. The incidence of epignathus is between 1:35,000 and 1:200,000 live births with a female predominance. We reported an uncommon case of epignathus in a female newborn baby with an ill-defined oral mass protruding through a cleft in the hard palate. Computed tomography scan showed a contrast-enhanced solid mass with areas of calcification simulating a unique case of maxilla duplication. Surgery was performed, the mass was excised successfully, and microscopic analysis confirmed the diagnosis of mature teratoma. The patient evolved with good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible, especially to avoid fatal airway obstruction. In such cases, the treatment option is exclusively surgical, and complete resection is curative in most cases during the early neonatal period.