Ten patients with arteriovenous malformations (AVMs) predominantly involving the lateral ventricle were operated upon. Computed tomography demonstrated intraventricular hemorrhage in eight patients, intracerebral hemorrhage large enough to warrant evacuation in two patients, and hydrocephalus in one patient who was admitted to the hospital because of generalized seizure. Cerebral angiography demonstrated an AVM in eight patients, but did not visualize the lesion in two patients. One patient suffered a recurrent intraventricular hemorrhage when the AVM was demonstrated, although repeated angiography had failed to disclose a vascular lesion at his first intraventricular hemorrhage 14 months before. Four AVMs were located in the head of the caudate nucleus; three were resected through frontal transcortical transventricular approach and one was resected through anterior transcallosal approach. Two of the former and the latter had excellent results. Two AVMs located in the temporal horn and trigon in the dominant hemisphere were excised through middle temporal gyrus approach with excellent results. Four AVMs of the dorsal surface of the thalamus were resected through posterior transcallosal approach; two had excellent results, and one had good result. We emphasize that the brain incision should be as small as possible when entering the lateral ventricle. Although various approaches have been proposed for lesions of the lateral ventricle, we recommend transcallosal approach for AVMs of the lateral ventricle. The position of the patient is very important; when the lateral ventricle is entered to excise on AVM, whether anterior or posterior transcallosal approach is used, the side of the lesion should be positioned slightly downward, so that the gravidity of the brain will aid in retracting the brain.