Coronary artery ectasia (CAE) is a rare form of aneurysmal coronary heart disease. It is defined as a dilatation of the coronary artery by more than one-third of its length and with a diameter 1.5 times of a normal coronary artery adjacent to it. This condition increases the risk of angina pectoris and acute coronary syndrome. Hence, we discuss the pharmacologic options for primary and secondary prevention of CAE complications. Antiplatelets such as aspirin are considered the mainstay of treatment in patients with CAE. Anticoagulants such as warfarin are warranted on a case-by-case basis to prevent thrombus formation depending on the presence of concomitant obstructive coronary artery disease and the patient's risk of bleeding. Since atherosclerosis is the most common cause of CAE, statins are indicated in all patients for primary prevention. Angiotensin-converting enzyme (ACE) inhibitors may be indicated, especially in hypertensive patients, due to their anti-inflammatory properties. Beta-blockers may be indicated due to their antihypertensive and anti-ischemic effects. Calcium (Ca) channel blockers may be needed to prevent coronary vasospasm. Nitrates are generally contraindicated as they may lead to worsening of symptoms. Other antianginal medications such as trimetazidine can improve exercise tolerance with no reported adverse events in these patients.