Teratomas of the head and neck are uncommon. They are composed of tissues from all three germ layers with varying degrees of differentiation. They arise from pluripotent stem cells and ectopic embryonic nongerm cells. Head and neck teratomas most commonly emerge during the neonatal period and are associated with airway obstruction and high rates of mortality in untreated patients. The tumors are usually benign, but when found during adulthood, they have a high incidence of malignancy. The most common site of occurrence is the cervical region and the nasopharynx. We presented a rare case of teratoma originating from the base of the tongue. This case is an example of the development of teratoma by pinching off of remnants of the three germinal areas, which become marginated in the normal migratory pathway as the different structures of the head and neck develop. The remnants developed into the predetermined organ systems, giving rise to the heterotopic tissues encountered. The recommended treatment for head and neck teratomas is surgical excision. When a patient is experiencing respiratory difficulty, establishment of an airway is a priority.