Aneurysm of the vein of Galen is a rare congenital disease, with a generally poor prognosis. It represents less than 1% of cerebral arteriovenous malformations. The intrauterine diagnosis of this vascular anomaly has been facilitated by vascular exploration with pulsed and color Doppler sonography. According to some studies focused on prognostical criteria, the perinatal prognosis seems to be invariably compromised when signs of cardiac decompensation develop prenatally, knowing that early delivery may generate additional complications in the very premature infant. Medical treatment is inefficient, because of the large, persisting systemic shunt, so that pregnancy termination may be performed, after parental consent, in some cases demonstrating all signs of cardiac decompensation. On the other hand, infants with normal velocity waveforms and a low extent of the systemic shunt have a good extrauterine adaptation. A full clinical evaluation of the affected neonates at the end of the first week of live is now the best way to select the infants for intravascular embolization.