Nasal neuroglial heterotopia is a rare, congenital lesion, composed of mature brain and neural tissue that has isolated from the cranial cavity or spinal canal. It typically presents at birth or in early childhood. We describe a case of nasal neuroglial hetrotropia in a 14 year old boy who had a history of right nasal obstruction and discharge since birth. Anterior rhinoscopy showed an oval, elastic, smooth and uncompressible mass at the upper third of the nasal septum. He underwent surgery with a preoperative diagnosis of nasal cyst. Histologically, the lesion showed mature glial and neural tissue in a loose fibrous stroma. The glial tissue was strongly positive for immunohistochemical (IHC) antibody Glial Fibrillary Acidic Protein (GFAP). The neural tissue was positive for S100 antibody. We are reporting the case because of its rarity, particularly in teen age, and to emphasize the importance of immunohistochemistry in reaching a correct diagnosis.