Background: Dyke-Davidoff-Masson syndrome is a rare condition resulting from brain injury due to a multitude of causes; especially in early life. Characteristics include cerebral hemiatrophy/hypoplasia, contralateral hemiparesis, seizures, and compensatory osseous hypertrophy. Case presentation: We present a case of a 14-year-old girl who initially presented with left sided hemiparesis. Computed tomography (CT) showed evidence of right cerebral atrophy. Workup for paediatric stroke was negative. Upon further evaluation by Neuroradiology, features suggesting Dyke-Davidoff-Masson syndrome were confirmed. Patient has been under Neurology follow up since. Conclusions: Due to its rarity, Dyke-Davidoff-Masson syndrome may easily be missed by the majority of treating clinicians. Knowledge of its features on imaging enables timely and accurate diagnosis – allowing appropriate management.