A case of incontinentia pigmenti achromians associated with mental retardation, epilepsy, short stature and ocular anomalies was reported. A study of this case together with a review of the 38 cases in the literature revealed that this entity has been associated with central nervous system involvements at a high frequency. It is necessary, therefore, to consider incontinentia pigmenti achromians as a neurocutaneous syndrome from the viewpoint of pediatric neurology.