Dyke-Davidoff-Masson syndrome is characterized by unilateral atrophy of cerebral hemispheres, enlargement of the ipsilateral sulci, ventricles, and cisternal spaces, decrease in the size of ipsilateral cranial fossae, overdevelopment of paranasal sinuses and mastoid air cells, and unilateral thickening of the skull. This syndrome is usually seen during adolescence; however, it can also be seen in childhood. We present a case of Dyke Davidoff-Masson-syndrome with typical clinical and imaging features.