Dyke–Davidoff–Masson syndrome (DDMS) is a rare disorder characterized by recurrent seizures, facial asymmetry, contralateral hemiplegia, radiologic features of cerebral hemiatrophy, and ipsilateral compensatory hypertrophy of the skull bone and sinuses due to cerebral injury that may occur in early childhood or fetal life. We describe two cases of DDMS, who initially presented with refractory seizures to the departments of Pediatrics and Medicine at Sri Devaraj Urs Medical College and Hospital. First case is of a 12 year old male with right hemiparesis and seizures since 3 months, CT & MRI brain revealed diffuse mild atrophy with paucity of white matter in the left cerebral hemisphere involving ipsilateral thalamus and midbrain, left cerebral hemiatrophy and chronic left MCA infarct with adjacent gliotic changes. Another case is of 35–year–male referred for right hemiplegia and uncontrolled seizures for 5 years, CT brain revealed thickening of cranial vault, pneumatosis dilatans of bilateral frontal sinuses, left cerebral hemiatrophy, and right cerebellar infarct and pneumatoencephalographic changes. In each case, the clinical features were noted along with computed tomography or magnetic resonance imaging which helped in confirming the diagnosis of DDMS. Therefore DDMS should also be considered as a differential diagnosis of refractory seizures.