Introduction: Vein of Galen malformation is a rare congenital cerebral Arteriovenousmalformation with low resistance intracranial shunting which results heart to generate a high cardiac output in order to maintain systemic perfusion and eventually leads to a decompensated state with increased pulmonary hypertension. Case presentation: We report a day-one male neonate referred for further management at birth and he was tachycardic with a hyperdynamic precordium, bounding pulses, a continuous machinery murmur, and significant pre- and post-ductal oxygen saturation differences. Findings suggested high-output right heart failure with warm, well-perfused extremities.Cranial examination revealed a bulging anterior fontanelle with an audible bruit. He developed refractory seizures, and therapeutic hypothermia was initiated for neuroprotection. Severe persistent pulmonary hypertension was treated with intravenousmilrinone, with dopamine and dobutamine for inotropic support. Cranial ultrasound on day one demonstrated a cerebral venous malformation measuring 1.6×1.7×2.1 cm in the posterior thalamic region with adjacent parenchymal haemorrhages. Echocardiography showed prominent superior vena cava flow , a large patent ductus arteriosus, dilated internal jugularveins, and severe pulmonary hypertension with a tricuspid regurgitation gradient of 60 mmHg. The Bicêtre score was below 8, indicating instability for intervention. Despite maximal medical therapy , the neonate died on day ten following massive pulmonary haemorrhage . Conclusion: Clinical recognition in this exceptionally rare, antenatally undetected cases maybe difficult, necessitating meticulous bedside examination and evaluation. Poses significant challenges in managing the complications and definitive treatment would be staged endovascular embolization of the shunt. No definitive consensus reached worldwide related to the timing of neurosurgical interventions.