Ectopic neuroglial tissue, or heterotopic brain tissue, presents a rare congenital condition characterized by the presence of benign neuroglial cells outside the central nervous system. Diagnosing neuroglial heterotopia solely based on clinical examination and radiological tests can be challenging, necessitating confirmation through histopathologic examination. These lesions may mimic other conditions such as dermoid cysts, pilar cysts, or lipomas. Here, we report a case of a 19-month-old boy with a mass lesion on the dorsum of the nose since birth. The lesion was initially suspected to be a congenital dermoid cyst, and the patient underwent surgical excision under general anesthesia. However, histopathological examination revealed it as an isolated nasal septum neuroglial heterotopia.