A Meningoencephalocele is an extracranial herniation of the meninges, CSF, and some portion of the brain, so a cranial defect is always present. The meningoencephalocele may be congenital or acquired in trauma and classify according to the location. The transethmoidal meningoence-phalocale is rare but important lesion that causes some characteristic and some misleading. A 4 year-old male patient had a transethmoidal meningoencephalocele with CSF rhinorrhea since 3rd month after birth. We report this case with review of literatures.