Teratomas are rare congenital neoplasms that arise from pluripotent cells. They consist of tissue from all three embryonic germ layers. According to most studies, teratomas of the head and neck account for only 2 to 9% of all cases. In this article, we report the first case of a teratoma of the nasal vestibule in a newborn. We also discuss methods of diagnosis and treatment of upper airway teratomas, and we include a brief review of the literature.