Interrupted aortic arch (IAA) is characterised by a lack of luminal continuity between the ascending and descending thoracic aorta. It represents a critical ductus dependent congenital heart disease, which without surgery is associated with high mortality in the neonatal period. Management remains challenging, although in recent years overall mortality appears to be improving. Long-term morbidity and need for repeated interventions are areas of concern. It is difficult to make generalised recommendations based on this literature review as complex associated anomalies often require individualised management strategy.