This article presents ten children with agenesis of the corpus callosum diagnosed during the period 1978-1992. The children were re-examined in 1992, and the results of the examination compared with the results of other authors. Among the ten children, five had other cerebral anomalies, six had eye anomalies, six were mentally retarded and two suffered from epilepsy. There was one boy with inversion on chromosome no. 1, one girl with Aicardie's syndrome, two sisters with agenesis of the corpus callosum (ACC), one of whom was suffering from diabetes insipidus, and two brothers with Walker Warburg's syndrome. ACC is often associated with mental retardation, epilepsy and eye anomalies and may be found associated to many different syndromes.