The vein of Galen aneurysmal malformation (VGAM) is an extremely rare arteriovenous malformation.The VGAM clinical manifestations vary depending on the magnitude of vascular compromise and the age at initial presentation.Neonates typically present with severe congestive heart failure.Here we present a case in which a systolic heart murmur was the first manifestation of high output heart failure due to a VGAM.