The following review of the literature describes the ganglioglioma, an uncommon mixed glioneuronal neoplasm, most often of low-grade histology, with a small, albeit well-documented, malignant potential. These tumors exhibit a strong epileptogenic propensity and most often present as new onset seizures or are discovered after a long history of refractory epilepsy. Despite their indolent course, the importance of gross total resection is well recognized to prevent anaplastic and malignant degeneration. Morphologically, the neoplasm is often cystic with an enhancing mural nodule, but can also be entirely solid. They are most often found in the temporal lobe but have been found throughout the neuraxis. An exceedingly rare location of the ganglioglioma is within the lateral ventricle. A systematic literature search revealed only eight reports documenting the occurrence of a ganglioglioma within the lateral ventricle. We describe an illustrative case of an intraventricular ganglioglioma with a prominent cystic component and enhancing mural nodule, which represents the classic radiographic appearance of gangliogliomas described in other locations. A superior parietal lobule approach offered excellent surgical access for tumor removal and the patient has remained free of neurological deficits following surgery. Regardless of location within the central nervous system, ganglioglioma should be on the differential diagnosis for any cystic mass with a mural nodule, particularly in the setting of epilepsy.