Dyke-Davidoff-Masson syndrome (DDMS) is a clinical condition characterized by epilepsy, hemiplegia or hemiparesis, mental retardation, facial asymmetry, psychiatric disorders, sensorineural hearing loss, cerebral atrophy on neuroimaging, excessive enlargement and air increase in the paranasal sinuses, 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 structures, 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.