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Congenital coronary anomalies present with a frequency of 0.7% in the general population. Most coronary anomalies are benign, but some may be linked to ischaemia and sudden cardiac death. In this case report, we describe the findings of a middle-aged man who was seen for unspecific cardiac symptoms. Echocardiography revealed a vascular abnormality recently recognised as an indicator of coronary anomaly, the retroaortic anomalous coronary sign. With this case, we wish to spread awareness of this sign to highlight what it indicates and what its potential implications are.