Nasal glioma, which is also known as nasal glial heterotopia, is a rare benign congenital lesion. A lot of explanations for the pathogenesis of this disease have already been provided. However, all of them lack theoretical basis. Nowadays, for nasal glioma, complete resection of the tumor is generally used in clinic treatment. CT examination or MRI is necessary for confirming the lesions and the relation between the tumor and intracranial part. This paper reported a neonatal nasal glioma case associated with congenital nasal deformity.