Nasal gliomas are rare, benign, congenital midline tumors made up of heterotopic neuroglial tissue. They have the potential for intracranial extension. They are commonly seen in newborns and children, and rarely in adults. Preoperative diagnostic imaging is essential to delineate the exact location, limits, and extensions of the tumor and thus to determine the appropriate surgical approach. Endoscopic surgery is considered appropriate for the removal of an intranasal glioma without intracranial extension. We describe a rare case of nasopharyngeal glioma in a newborn who presented with early respiratory distress, and we include a review of the literature.