The authors report a case of intracranial extracerebral glioneuronal heterotopia (IEGH) in an infant. Glioneuronal heterotopias are rare congenital disorders that arise in the head, face, spine, and thoracic cavity. They consist of nodular accumulations of neuronal and glial cells that have developed abnormally, ranging in size from small lesions to large masses. Among heterotopias, IEGHs are relatively rare. They cause various clinical symptoms, depending on their size and location. The neuroimaging studies, histological examinations, and intraoperative findings presented provide insight into the pathogenesis of this disorder. The findings support the separation and detachment theory, which proposes that IEGHs originate from a third telencephalon that erroneously forms between the 4th and 6th week of embryogenesis. More detailed case reports are necessary to understand fully the pathogenesis of IEGHs.