The term jaw tumor of embryonic origin includes vestiges of organogenetic processes, hamartomas, teratomas, and blastemal tumors. Teratomas of the head and neck account for less than 2% of all teratomas. Epignathus is a subtype of congenital facial teratoma affecting the maxilla. It is a severe fetal pathology which causes deglutition disorders and severe hydramnios and may lead to therapeutic abortion. Oral cavity teratomas are almost always mature and thus benign and encapsulated. They may have an impact on maxillary growth. Surgery is the only treatment. It can be immediate postbirth tumor removal when neonatal respiratory distress can be managed by the anesthesiologist, or an EXIT (ex utero intrapartum) procedure.