Anomalous origin of the coronary arteries is rare in clinical practice. Different anomalies have different clinical or symptomatic manifestations depending on the patient’s age. However, the majority of coronary artery origin anomalies are silent and are only discovered incidentally during Left Heart Catheterization (LHC). Acute Myocardial Infarction (MI) or even sudden death may occur in patients with an anomalous origin of the Left Main (LM) coronary artery from the right coronary cusp with a subsequent course between the aorta and the pulmonary artery. Therefore, recognition of anomalous origin of the coronary arteries is essential for clinical practice to further classify and properly manage patients with increased risk of Acute Coronary Syndrome (ACS). Furthermore, patients found to have an anomaly may be eligible for surgical correction.