Coronary artery anomalies have been identified in 0.6–1.5% of coronary angiography (CAG) studies . The incidence of left circumflex coronary artery (LCX) anomaly was reported as 0.3% . An anomalous origin of the coronary artery is a rare congenital condition with an incidence of about 0.17% based on autopsy cases . The ostium of an aberrant vessel may be difficult to identify in the diagnostic procedure, but recognition and adequate visualization of the anomaly are essential for appropriate patient management, especially in patients undergoing evaluation for percutaneous coronary intervention (PCI) and cardiac surgery [3, 4]. We report the case of congenital absence of the LCX with a dominant right coronary artery (RCA) in a 68-year-old man with acute inferior myocardial infarction (MI).