Dyke-Davidoff-Masson syndrome (DDMS) is a rare, but important cause of drug-resistant seizures. Dyke-Davidoff-Masson syndrome is a constellation of clinical features that consists of hemiparesis, seizure, facial asymmetry, and intellectual disability with distinct neuroimaging features. A 27-year-old lady presented to us with drug-resistant epilepsy, hemiparesis, and intellectual disability that necessitated her withdrawal from school. Her brain magnetic resonance imaging (MRI) showed cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses consistent with DDMS. We discuss the diagnostic and therapeutic implications of DDMS and advocate early referral and evaluation of people with epilepsy in sub-Saharan African settings.