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Coronary artery aneurysm is a rare congenital or vascular inflammation-based anomaly for which the clinical course and optimal timing of treatment remain unclear. Here, we report a case of sudden death caused by a giant coronary artery aneurysm of the left anterior descending artery that presented with chest pain. This case suggests that urgent interventional or surgical repair is needed when a large coronary aneurysm presents with acute ischemic symptoms.