Coronary artery ectasia (CAE) is one of the uncommon cardiovascular disorders. Its incidence ranges from 1.2%-4.9%. Coronary artery ectasia likely represents an exaggerated form of expansive vascular remodeling (i.e. excessive expansive remodeling) in response to atherosclerotic plaque growth with atherosclerosis being the most common cause. Although, it has been described more than five decades ago, its management is still debated. We therefore reviewed the literature until date by searching PubMed and Google scholar using key words “coronary artery ectasia”, “coronary artery aneurysm”, “pathophysiology”, “diagnosis”, “management” either by itself or in combination. We reviewed the full articles and review articles and focused mainly on pathophysiology, diagnosis and management of CAE.