We report a 1 day old girl with a nasal mass that was diagnosed as an intranasal glioma. The tumor mass was noted to be localized laterally from, and based along, the inferior turbinate. Nasal glioma is a developmental abnormality of neurogenic origin with no malignant potential. An intranasal mass requires careful rhinological, neurological and radiological examinations. With adequate initial removal, excisional biopsy usually allows complete recovery. A frontal craniotomy approach is preferred for those patients who have nasal glioma with an intracranial connection. With no evidence of an intracranial connection, a conservative extracranial approach is recommended.