Dyke-Davidoff-Masson syndrome (DDMS) is a clinical condition characterized by epilepsy, hemiple-gia or hemiparesis, mental retardation, facial asymmetry, psychiatric disorders, sensorineural hearing loss, cerebral atrophy on neuroimaging, excessive enlargement and air increase in the paranasal si-nuses, and unilateral skull thickening. In this paper, a 16-year-old male patient who was followed up with diagnoses of epilepsy, intellectual disability, and left hemiparesis, and who was diagnosed with DDMS upon the detection of right cerebral atrophy, thickening in the right hemiclavarial bone structu-res, and increased aeration of the frontal sinuses on neuroimaging, is presented. DDMS, a rare case in the literature, is emphasized for consideration in the differential diagnosis of cerebral hemiatrophy.