Congenital infantile midline nasal meningoencephaloceles are rare anomalies. A 5-month-old Egyptian boy otherwise healthy presented with history of respiratory distress, difficulty in oral feeding and appearance of a mass in right nostril. The CT scan showed soft tissue density lesion filling the right nasal fossa with small bony defect in the most anterior part of anterior cranial fossa adjacent to right side of the crista galli suggesting meningoencephalocele. MRI showed the same mass criteria as on CT with fluid signal intensity. The lesion was approached and removed endonasally under general anesthesia with the use of different angles endoscopes. The mucoperiostium covering the posterior two third of the ipsilateral inferior nasal turbinate was harvested and used for repair of the defect. The patient was discharged from the hospital after 48h of surgery and was followed up for 3 months after surgery with no reported symptoms or related surgical complication. To the best of our knowledge, this is the youngest patient reported in the literature to be treated successfully with this approach.