A case report of an 11 years old boy with new onset of a seizure disorder is presented. A computed tomography scan demonstrated a noncalcified, nonenhancing focal region of abnormal cortex. A magnetic resonance imaging scan delineated both an isointense area of abnormally thickened gyri and linear areas of abnormal high signal intensity in the subjacent white matter. A review of the radiologic and pathologic literature suggests that this lesion represents the entity focal cortical dysplasia as described by Taylor, et al. This abnormality is part of a spectrum of disorders including hamartomas (of tuberous sclerosis), focal cortical dysplasia and heterotopias.