Vein of Galen malformations (VGMs) are rare, consisting approximately 1% of intracranial vascular malformations, but nearly one third of pediatric vascular malformations, which can cause severe morbidity and mortality in infants and older children, but more particularly in neonates. We presented 2 patients with VGMs which were confirmed by characteristic clinical symptoms and neuroradiological findings (including CT, MRI and/or angiography). One patient underwent embolization followed by CT, MRI and conventional angiography. The patient’s neurological and cognitive status was normal at present. The other neonate died of congestive heart failure 36 h after birth.