Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder found in children as well as adults. It is characterized by hemi cerebral atrophy. To date, very few cases of this disorder have been reported. Radiological imaging including magnetic resonance imaging (MRI) and computed tomography (CT) are accurate tools for the diagnosis of DDMS. We present a case of a 13-year-old female child who came with complaints of multiple episodes of generalized tonic-clonic seizures. In our case, clinical history and imaging with CT and MRI were accurate enough to diagnose DDMS.