Congenital nose neoplasms are infrequent, between them neurogenic tumors of the middle line include nasal gliomata (glial ectopies) and nasal encephaloceles, according to an existing or lacking communicating link with the intracranial cavity. We report one congenital naso-encephalocele case in a 16-year-old girl suffering from repeated meningitis events after several nasal polypectomies performed in other departments. She underwent complete removal through fronto-neurosurgical and paralateronasal approach, being the young woman asymptomatic for 3 years. We discuss about both intranasal gliomata and encephaloceles, certainly of not easy pathologic identification, stressing the decisive value of imaging techniques, as magnetic resonance, in order to clearcut both the diagnosis and the therapeutical planning.