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Aim of the study: The aim of this case report is to outline the difficulties associated with managing a child with nasal glial heterotopia. Developmental midline nasal mass, especially of neurogenic origin, poses a great challenge to managing surgeons. Nasal glial heterotopia is among benign congenital midline nasal tumours with aggressive features. Case report: Herein, we report the case of a 5-month-old infant who presented earlier, at 3 months old, with respiratory distress secondary to obstructive nasal mass. Result and discussion: Imaging and bedside flexible nasopharyngolaryngoscopy showed a localised nasal mass with no intracranial tumour. Following two combined endoscopic intraoral and transnasal tumours, the diagnosis of nasal glial heterotopia was made, and the patient is under surveillance. Conclusion: Multidisciplinary team discussion along with combined endoscopic transnasal and transoral approach ought to be considered in managing challenging congenital nasal tumours in children.