True malformations of the vein of Galen are usually diagnosed within the first weeks of life. They represent less than 1 p. 100 of the cerebral arteriovenous malformations. These true malformations are exceptional in adults but should be known as an endovascular treatment could be performed. The most frequent clinical presentation is a severe cardiac failure leading to death. We report a giant arteriovenous malformation of the vein of Galen in a right-handed, 50 year-old man. During childhood, he suffered from a compensated cardiac failure which remained of unknown cause. Neurological examination showed kinetic and static cerebellar syndrome and a Parinaud syndrome. A cerebral MRI scan revealed a giant vascular malformation of the vein of Galen with a normal posterior fossa. The angiography enabled the diagnosis of a true malformation of the vein of Galen in its choroidian form. Its high blood flow entails cardiac failure because of a steal phenomenon. An endovascular treatment was declined because of numerous arterial afferences and the potential risks of peroperative haemorrhage.