Dyke-Davidoff-Masson syndrome (DDMS) is usually described secondary to prenatal or perinatal insult and is characterized by seizures, cerebral hemiatrophy, hemiparesis/facial asymmetry, and learning disability. We report an adolescent-onset case who presented with drug-refractory seizures and was considered for hemispherotomy on the dominant side. This is the first case report of DDMS where Wada helped in lateralizing the language to the ipsilateral dominant hemisphere despite the atrophy of the left inferior frontal gyrus. Thus, Wada is recommended in all patients without language regression who are considered for dominant hemispherotomy.