Vein of Galen arterio-venous malformation is a rare congenital anomaly, which presents in the neonatal period. High output heart failure in the absence of an underlying structural heart defect points towards the condition and is an important contributing factor for morbidity and mortality. We describe a case of a term newborn who presented with respiratory distress and cardiomegaly. The characteristic echocardiographic findings of high output cardiac failure were found and eventually led to the diagnosis of an underlying vein of Galen arterio-venous malformation. The relevant literature is discussed.