Coronary artery anomalies (CAAs) are rare, occurring in less than 1% of the global population. Although they are often discovered incidentally, CAAs can be a cause of sudden death. They are a leading cause of cardiac death in young athletes, thus, necessitating careful monitoring of this group. Advances in diagnostic technology and cardiac imaging, including transthoracic and transesophageal echocardiography, as well as other cardiac imaging modalities, are recommended for more accurate assessment when a coronary anomaly is suspected. The commonest form of coronary artery disease is caused by atherosclerosis with its known phenotypic presentation in the form of thickened intema media and plaque formation. Severe cases may present with obstructive coronary artery disease of one or more of the epicardial branches. The clinical presentation varies between acute coronary syndrome and stable angina. Even, myocardial infarction may present with target lesions or non-obstructive coronary disease. Finally long standing coronary artery disease may affect myocardial function with significant ventricular remodelling and related comnplications. Echocardiography with all its modalities, transthoracic, transesophagea, stress echo and intravascular ultrasound play different important roles in the management of individual patients.