A 36-hour-old term neonate presented with refractory cardiogenic shock and multi-organ dysfunction. On suspecting coarctation of aorta, intravenous prostaglandin was started but without any benefit. Echocardiography revealed isolated anomalous origin of right pulmonary artery from ascending aorta causing massive aortic steal, supra-systemic pulmonary arterial hypertension (PAH), multi-organ dysfunction and right ventricular failure. The main pulmonary artery was solely continued as left pulmonary artery. Emergency surgery at 48 h was life-saving and mid-term follow-up is excellent. The authors present this case as an important alternative diagnosis to duct dependent systemic lesions. The English literature in the last two decades was reviewed to discuss the characteristics, the diagnosis and treatment of neonatal presentation of this rare anomaly. Incidentally, the reported case is the youngest successful survivor of this surgery.