Gangliogliomas are mixed tumors consisting of both glial elements and differentiated neurons. Although any part of the central nervous system can be affected, little is known about intraventricular gangliogliomas. A patient with a ganglioglioma is presented in the previously unreported location of the anterior third ventricle at the foramen of Monro, mimicking a colloid cyst. We review all other reported cases of intraventricular ganglioglioma (n=6) to characterize this entity. Intraventricular gangliogliomas typically affect younger patients with female predominance (male:female, 2:5; median age 25 years). Symptoms occur secondary to obstruction of physiological cerebrospinal fluid circulation. Complete surgical resection with re-establishment of cerebrospinal fluid drainage is the goal of treatment.