We report two patients with medically refractory epilepsy who had MRI evidence of unilateral subcortical nodular heterotopia and agenesis of corpus callosum. The abnormal hemisphere was small, whereas the contralateral normal hemisphere appeared large and crossed the midline. Although the normal hemisphere was initially mistaken for hemimegalencephaly, there were no typical radiological features. Moreover, the electroencephalographic abnormalities lateralized to the hemisphere showing heterotopia. Because contralateral hemispheric abnormalities like heterotopia, hemimicrencephaly, and hemimegalencephaly can occur in patients with hemispheric heterotopias, we emphasize the importance of careful scrutiny of the contralateral hemisphere in patients with unilateral heterotopia. Absence of typical radiological features and appropriately lateralized electroencephalographic abnormalities will help differentiate the two. This is crucial when planning epilepsy surgery.