Isolated complete corpus callosal agenesis is a rare entity. Usually this condition will be an associated finding in other syndromes. 3 month old male child came with complaints of deformed foot on both sides, not having a social smile and neck holding. Patient referred to the Radiology department for MRI brain which showed complete absence of corpus callosum, widely separated and parallely placed lateral ventricles, colpocephaly, high riding of 3rd ventricle and absence of cingulate gyrus and radial arrangement of gyri along the interhemispheric fissure. Hence it was reported as isolated complete corpus callosal agenesis and this article describes the Embryogenesis, anatomy, developmental anomalies and its clinical manifestations & prognosis.