✓ In order to determine adequate therapeutic approaches for cavernous malformations of the third ventricle, the authors reviewed a series of five such malformations managed at their institution and nine others reported in the literature. Four subgroups were identified in terms of the site of origin and could be characterized by different clinical manifestations: visual field defects and endocrine function deficits in patients with malformations in the suprachiasmatic region (six cases); symptoms caused by hydrocephalus in those with malformations in the foramen of Monro region (five cases); and deficits of short-term memory in those with malformations in the lateral wall (two cases) or of the floor of the third ventricle (one case). Unlike cavernous malformations at other locations, malformations of the third ventricle frequently demonstrated rapid growth (43%) and mass effects (71%). The surgical or autopsy findings suggested that the growth was attributable to repeated intralesional hemorrhages. Extralesional hemorrhage was also not uncommon, occurring in 29% of patients. Such tendencies require the adoption of a more aggressive approach to this particular group of cavernous malformations as compared to those in other locations. The risks of regrowth and extralesional hemorrhage appear to be reduced only by complete excision. The surgical approaches adopted should be aimed at providing the best access to the site where the malformation has arisen. The translamina terminalis approach for cavernous malformations in the suprachiasmatic region, the transventricular or transcallosal interfornicial approaches for those in the foramen of Monro region and the transvelum interpositum approach for those in the lateral wall or the floor of the third ventricle appear to be appropriate. In order to select the adequate surgical approach, precise diagnosis of the site of origin is crucial. In addition to neuroimaging techniques, the patient's initial symptoms provide valuable information.