Nasal encephaloceles can be divided into frontoethmoidal and basal encephaloceles. Both conditions are very rare, but frontoethmoidal encephaloceles show a relatively high incidence (1:5,000) in Southeast Asia. The pathogenesis of encephaloceles may be explained by a disturbance in separation of surface ectoderm (epithelial layer) and neurectoderm (nervous tissue) in the midline just after closure of the neural folds. It should be regarded as a 'late' neurulation defect taking place during the 4th gestational week. Apoptosis appears to be related to this separation process. Frontoethmoidal encephaloceles can be recognized as a facial mass covered with normal skin, while basal encephaloceles may cause nasal obstruction or symptoms related to herniation of basal structures. Diagnostic CT or MR imaging delineates the anatomy of the herniated mass. Therapy for frontoethmoidal encephaloceles consists in excision of the cele, watertight closure of the dural defect and reconstruction of the skull defect. Basal encephaloceles may harbour vital herniated structures which should be saved. Hydrocephalus should be dealt with first, followed by elective single-stage reconstructive surgery. The prognosis appears to be better for patients with frontoethmoidal encephaloceles than for patients with occipital or parietal encephaloceles, and it depends largely on the presence of additional congenital anomalies of the brain. The differential diagnosis of a nasal mass must include nasal glioma, dermoid cyst, and nasal polyp.