Background: Gangliocytomas are rare neuronal tumors with an incidence of less than 1% of all CNS neoplasms. They are mostly seen in the pediatric age group, localizing within the cerebral cortex, most often the temporal lobe. Methods: We report a case of an intracranial gangliocytoma arising within the lateral ventricle in an adult patient. Our 66-year-old female patient started experiencing progressive generalized weakness, headaches, and confusion prior to presentation. Her neurological examination did not reveal any focal neurological deficits. MRI of the brain showed a diffusely enhancing lobulated mass situated within the frontal horn of the right lateral ventricle with extension into the foramen of Monro and obstructive hydrocephalus. Results: The patient underwent an interhemispheric trans-callosal approach with gross total resection and relief of her hydrocephalous. Pathological examination showed clusters of highly pleomorphic neuron-like cells without evidence of neoplastic glial cells. Histopathological and immunohistochemistry findings were consistent with the diagnosis of gangliocytoma (World Health Organization grade 1). Conclusions: Gangliocytomas are rare low-grade CNS neoplasms that can present in an older population within unusual locations and should be included within the differential whenever a suspicious lesion is encountered.