A neonate with cyanosis at birth was found to have a rare type of tetralogy of Fallot. Echocardiography showed dextrocardia, left aortic arch with constricting and tortuous patent ductus arteriosus (PDA). Computed tomography angiography thorax revealed visceroatrial situs inversus, mirror image dextrocardia, tetralogy anatomy and tortuous right PDA arising from bovine brachiocephalic artery. In view of severe cyanosis, emergency division of PDA and modified right Blalock-Taussig shunt through median sternotomy were performed under cardiopulmonary bypass. Post-operatively, the sternum was left open and he developed pulmonary overcirculation requiring prolonged ICU stay. He was discharged well at Day 26 post surgery. This case highlights a rare association tetralogy of Fallot, dextrocardia and situs inversus, with concomitant unilateral right PDA and bovine aortic arch.