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Conclusion This issue should be explored in prospective studies in larger groups.Coronary artery anomalies (CAAs) are congenital disorders with multiple variations in the number, shape, and location of the Ostia of the coronary arterial system. The congenitally absent left main coronary artery (LMCA) is a rare anomaly that can present with benign or fatal complications ahead in life. Diagnosis and management of CAAs are sometimes challenging in low-risk patients. We present a unique case report of a 69-year-old Hispanic female who presen