Frontoethmoidal encephalomeningocoele is a rare congenital disease in which an intracranial mass protrudes through a midline defect from the anterior cranial fossa into the facial skeleton. The condition affects patients in South East Asian countries, such as Thailand, Burma, Malaysia and Indonesia, with frequency of 1 in 5000. The pathogenesis of encephalocoeles may be regarded as a 'late' neurulation defect during the fourth gestational week. We present a case of frontoethmoidal encephalomeningocoele with corpus callosal agenesis and colpocephaly; this may well be the first report of this combination. The patient had a bulging mass in the middle frontonasal area, with broadening of the nasal bridge and hypertelorism. Computed tomography scans delineated the skull defect and associated brain anomalies. A one-stage, combined transfacial-transcranial approach, correctional procedure was performed. We present here a discussion of the findings, with special reference to the condition's pathogenesis, morphological classification and evolving surgical treatments. Early diagnosis and referral, involving multidisciplinary teamwork, are of paramount importance because of the distorting influence of the extruding mass on facial growth.