The corpus callosum is composed of 4 segments namely the rostrum, thegenu, the body and the selenium 20 weeks all of these components are expected to be present in the midline. It continues to grow in thickness and length and reaches maturity around the age of 9-10 months [1]. Agenesis of the Corpus Callosum (ACC) is a heterogeneous congenital neurodevelopmental disorder that can be seen in isolation or in combination with other brain abnormalities such as are Dandy walker Disease, Mega large cistern, vermal hypoplasia and Rathke’s pocket cyst [2]. Then, ACC is dysgenesis of the callosum which results in the complete or partial absence of its components. The MRI is the gold standard of Diagnosis, shows the frontal horns of the lateral ventricles which take a disposition having an aspect of the horns of bulls [1,3]. This typical appearance when present is pathognomonic for ACC as showing in Figures 1A and 1B.