Eighteen cases of anterior encephalocele treated at the University of Malaya Hospital between 1970 and 1980 are discussed, and the literature concerning this defect is reviewed. A detailed analysis of the microscopic abnormalities present in the surgical specimens is included, along with the relevant radiographic and demographic data. Anterior encephalocele is more common in Southeast Asia than elsewhere. The possible ethnographic and geographic implications are presented, as well as a discussion of the relevant embryology, in attempting to define possible etiologies for this malformation. The author's surgical approach to the repair of this defect and reasons for preferring a transcranial, intradural approach are described. Potential complications are enumerated.