Mature teratomas, or teratoid tumors, or benign teratomas, of the nasal cavity and sinuses are exceptional the diagnosis is histopathological and shows within the lesion different types of tissue: fat, glial, muscular, cartilaginous, bone and respiratory. Teratomas are rare congenital neoplasms that originate from pluripotent cells. In this article, we report the first case of a nasal teratoma. We also discuss methods for diagnosing and treating upper respiratory teratomas, and include a brief literature review.