ABSTRACT BACKGROUND: Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease with unknown frequency, mainly presented withseizures, facial asymmetry, contralateral hemiparesis and mental retardation. Often, resulting from brain injury due to a multitude of causes, especially in early life, associated with birth asphyxia. Radiological findings include cerebral hemiatrophy/hypoplasia, calvarial thickening, and hyperpneumatization of the frontal sinuses. CASE PRESENTATION: We report the case of a 17-year-old male patient who presented to Neurology Clinic with complaints of leftside body weakness, walking difficulty and poorly controlled seizure for the past 6 years. Brain MRI revealed atrophy of the right cerebral hemisphere.CONCLUSION: Dyke-Davidoff-Masson syndrome should be suspected in any patients who present with classical features and brain imaging showing hemiatrophy. Early identification and treatment is important in such patients, as it can improve patients prognosis and quality of life.KEYWORDS:Dyke-Davidoff-Masson syndrome, Seizure, Cerebral hemiatrophy,Magnetic resonance imaging