A 35-year-old man developed meningitis for the first time at the age of 23. Thereafter, he repeatedly suffered from meningitis for six times before he was admitted to our clinic. Each meningitis was preceded by upper respiratory infection. Chronic infection in the head and neck, fracture of the skull base, liquorrhea, Mollaret meningitis, and acquired immunodeficiency were all ruled out. Nasal fiberscope examination revealed a palpating white tumor in the left olfactory cleavage. Coronal CT scanning demonstrated encephalomeningocele at the left ethmoidal cavity. The encephalomeningocele was surgically repaired and the patient has been free from further meningitis. The causal relationship between the encephalomeningocele and recurrent meningitis was discussed.