ABSTRACTIntroductionNasal glial heterotopia is a rare congenital developmental disorder characterized by meningeal epithelium and/or glial components.Case presentationA 2‐month‐old boy presented for treatment of a congenital mass in the right nasal cavity near the pharynx. The preoperative diagnosis was congenital intranasal neoplasm. Nasal endoscopic resection of the nasopharyngeal mass was performed under general anesthesia. Histological findings in the resected tissue supported a diagnosis of intranasal glial heterotopia. The surgical outcome was good and no surgical site infection occurred. During 1 year of follow‐up, the patient did not exhibit recurrence of heterotopia or related symptoms.ConclusionTransnasal endoscopic surgery is recommended for patients with intranasal glial heterotopia. Thorough preoperative imaging should be performed before glioma resection. The mass should be differentiated from encephalocele to prevent cerebrospinal fluid leakage and meningitis.