Introduction: Double aortic arch is a rare congenital vascular anomaly in which the aorta forms a complete ring around the trachea and oesophagus, resulting in airway compression. It is the most common type of complete vascular ring and often presents early in life with respiratory symptoms. Early recognition is essential, particularly in neonates with unexplained ventilatory failure and normal lung imaging. Case presentation: A term neonate developed inspiratory stridor within hours of birth,progressing rapidly to severe respiratory distress requiring endotracheal intubation and mechanical ventilation. Despite appropriate ventilator settings and confirmed tube position, the infant exhibited persistent carbon dioxide retention with respiratory acidosis, whileoxygenation remained relatively preserved. Chest radiography showed clear, well-expanded lung fields. As hypercapnia was disproportionate to radiological findings, extrinsic airway obstruction was suspected. Echocardiography suggested a double aortic arch with tracheal compression, along with an ostium secundum atrial septal defect and moderate tricuspid regurgitation. Aortic angiography and bronchography confirmed a dominant left aortic arch and a smaller right arch forming a complete vascular ring with tracheal indentation above the carina. Surgical division of the non-dominant arch was performed, resulting in marked clinicalimprovement and successful weaning from respiratory support. Conclusions: Double aortic arch should be considered in neonates presenting with early stridorand unexplained ventilatory failure, particularly when lung imaging is normal. Prompt diagnosis and timely surgical intervention lead to favourable outcomes.