The ring-sling complex is a rare developmental anomaly combining pulmonary artery sling and complete tracheal ring. The abnormal left pulmonary artery is a sling-like structure that surrounds the trachea and produces upper airway obstruction. Complete tracheal ring is associated with tracheal stenosis which often leads to respiratory distress. This article presents a case of this anomaly and emphasizes the importance of detecting the complex combination when evaluating related disease spectrum.