Teratomas are neoplasms composed of tissues from all three germ layers with varying degrees of differentiation. They are most commonly found in the sacrococcygeal and gonadal regions and rarely occur in the head and neck region. A teratoma is termed "epignathus" when it arises from the skull base or hard palate and is located in the oral cavity. The authors describe a case of a giant epignathus teratoma originating in the skull base of a neonate, extending bilaterally via two pedicles throughout the hard palate and protruding through the oral cavity. The tumor was completely resected using a transpalatal endoscopic endonasal approach. The excised tumor proved to be an immature teratoma with well-differentiated yolk sac elements. At the 1-year follow-up the patient showed no evidence of tumor recurrence and the child remains neurologically intact. This report demonstrates the use of a transpalatal endonasal corridor in a preterm infant. This approach provided an ample corridor into the ventral skull base without the need for external excisions and/or disruption of osseous elements.