We reviewed the diagnostic features and clinical outcome of 7 consecutive neonates who were diagnosed to have cerebral arteriovenous malformations. All presented with cardiac failure, and a cranial bruit was heard in 6/7 patients. There was electrocardiographic evidence of myocardial ischemia in 6 patients. The diagnosis was established at cardiac catheterization, or by cardiac and cranial ultrasound. Three patients died of heart failure before definitive treatment. Despite early intervention, three of the remaining four patients died either during or immediately after embolization or ligation of the fistula. A cerebral arteriovenous malformation is a rare cause of neonatal heart failure. Despite prompt recognition and aggressive treatment, the outlook for symptomatic neonates is poor.