Frontoethmoidal meningoencephaloceles constitute a well-defined clinical entity with remarkable epidemiologic peculiarities, being very prevalent in Southeast and Southern Asia, but relatively rare in Western Europe, Japan, Australia, and North America. They do not show an increased risk of recurrence in siblings and offspring, unlike other cephaloceles and neural tube defects generally, from which they should be distinguished. Combined craniofacial and neurosurgical operative treatment permits one-stage correction of the whole deformity, the associated hypertelorism being reduced by selective orbital translocation and the nasal deformity by rhinoplasty. In planning these procedures, three-dimensional CT scanning is very helpful. Although our experience is chiefly with cases referred in childhood, we have some experience with operations done in infancy and believe this to be the ideal age.