A rare type of congenital heart disease is an interrupted aortic arch (IAA), which affects approximately 1.5% of congenital heart disease patients. Interrupted aortic arch is an anomaly that can be considered the most severe form of aortic coarctation. In an IAA, there is an anatomical and luminal disruption between the ascending and descending aorta. IAA is a ductus dependent lesion since this is the only way the blood flow can travel to places distal to the disruption. This activity reviews the presentation of the interrupted aortic arch and highlights the role of the interprofessional team in its management.