Some individuals develop a rare form of coronary heart disease called coronary artery ectasia (CAE). It is characterized by a dilation of more than one-third of the length of a coronary artery and a diameter that is 1.5 times that of the adjacent normal coronary artery. In the absence of significant coronary constriction, angina pectoris, positive stress tests, and acute coronary syndromes may all be indications of CAE. A distal embolization, vasospasm, or vascular rupture may cause thrombus formation in an ectatic artery. Antiplatelets, such as aspirin, are the cornerstone of treatment for people with CAE. Anticoagulants are used to prevent thrombus formation based on the presence of concurrent obstructive coronary artery disease and the patient’s risk of bleeding. As atherosclerosis is the most common cause of CAE, all patients are advised to take statins for primary prevention. Due to their anti-inflammatory properties, angiotensin-converting enzyme inhibitors may be prescribed to individuals with hypertension. If hypertension and coronary vasospasm occur simultaneously, beta-blockers and calcium channel blockers may be beneficial. Because they may aggravate symptoms, nitrates are normally not suggested. Other CAE treatment methods include the prevention of thromboembolic complications and percutaneous or surgical revascularization. CAE prognosis is determined on the severity of the associated coronary artery.