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Background: Vein of Galen aneurysm (VGA) is the most common form of symptomatic cerebrovascular malformation in neonates. It develops in a fetus in the first trimester of pregnancy due to unknown reasons, but it is likely to have a genetic etiology. The prognosis of VGA is usually poor, particularly in newborns with heart failure due to high-flow intracerebral shunt. Surgery and endovascular embolization are partially successful treatments for controlling congestive heart failure and pulmonary hypertension. Case report: In this article, we present the case of a 3600-gram, full-term, female neonate hospitalized with respiratory distress and severe heart failure. The neonate was the second child of a 28-year-old mother. After echocardiography and brain sonography, she was diagnosed with the vein of Galen malformation. The case had severe and persistent congestive heart failure and refractory pulmonary hypertension. Conclusion: It is important to perform the auscultation of fontanel in newborns and consider VGA as a differential diagnosis in the neonates with congestive heart failure.