A broad array of tumors of the central nervous system may arise within the third ventricular region, including gliomas; subependymal giant cell astrocytomas; meningiomas; pineal region tumors; and cysts, including ependymal, arachnoid, epidermoid, and suprasellar tumors (i.e., macroadenomas, craniopharyngiomas, meningiomas, and optic gliomas). The clinical presentation varies with tumor location in and around the third ventricle. Tumors of the inferior portion of the anterior third ventricle can cause endocrine complaints, including diabetes insipidus and hypopituitarism as well as visual field impairment. Tumors of the superior portion of the anterior third ventricle result in obstructive hydrocephalus and cognitive memory deficits. Tumors of the posterior third often create outflow obstruction and hydrocephalus, and pineal region tumors are associated with Parinaud syndrome. Multiple surgical approaches may be considered in resecting tumors of the third ventricular region, including transcortical or interhemispheric transcallosal, interforniceal, transforaminal, orbitozygomatic subfrontal, or infratentorial supracerebellar. Each approach has strengths and weaknesses, and the choice is often made according to the surgeon’s experience and comfort level. The goals of surgery must be carefully considered so as to minimize neurologic morbidity or mortality. Finally, adjuvant chemotherapy and/or radiation therapy may play a significant role in treating tumor after tissue diagnosis.