Our approach to treating a patient with a vein of Galen aneurysm is, of course, influenced greatly by the age of the patient, the clinical symptoms, and the angiographic architecture of the malformation. Therapeutic options are primarily based on whether a true AVM is present or if the malformation represents an arteriovenous fistula involving the vein of Galen. Arterial endovascular approaches, microneurosurgery, and/or radiosurgery are preferred for management of the former; the transvenous endovascular approach has become the cornerstone of treatment in the latter. The most critical group, however, is the neonates in extreme cardiovascular distress. In this case our therapeutic intervention is initially endovascular from the venous side, either transfemoral or transtorcular. The immediate goal is to increase resistance to right ventricular output. Advantages of this approach over a transarterial approach include a shorter anesthesia time, minimal fluid and/or contrast administration, and creation of a wire "basket" or "bird's nest" on the venous side that helps prevent emboli that may be deposited on the arterial side in subsequent embolizations from passing through the malformation. The transvenous approach can be easily repeated multiple times and may be supplemented by transarterial embolizations. Endovascular coils have been the mainstay for such venous embolizations. The end point of treatment is not complete occlusion of the fistula but improvement in cardiac function. Often, more than one stage is required to reach our goal. The results in recent years have been encouraging and are to a large degree attributable to the advances in endovascular approaches. With future improved tools for diagnosis and treatment, perhaps the prognosis for this difficult malady also will continue to improve.