Intranasal encephalocele is known as a rare disease. Recently the author has experienced one case of intranasal encephalocele. This long standing congenital defect, with widening and flattening of the nasal bridge simulating hypertelorism, was successfully repaired via an extracranial approach. The procedure consisted of ligation and removal of the sac and lining the cavity with a frontal fascia. The patient was discharged with good condition on 16th hospital day and quite well up to day postoperative 3 months.