Dyke-Davidoff-Masson syndrome is a congenital, neonatal or early infantile condition characterized clinically by seizures, contralateral hemiplegia or hemiparesis, variable degrees of facial asymmetry, and mental retardation. Mental retardation is not always present and seizures may appear months or years after the onset of hemiparesis. We present two cases of this uncommon condition and discuss its imaging features, differential diagnosis, treatment options and prognosis.