Aneurysmal coronary artery disease is frequently encountered in clinical cardiology practice. Although more commonly associated with atherosclerosis, a variety of other acquired (eg, inflammatory, infectious, iatrogenic) or congenital causes have been identified. Recent research on the pathogenesis of coronary aneurysms has yielded interesting results. Advances in imaging have also provided new insights as to the nature of angiographic coronary aneurysms. Critical assessment of the abnormal flow dynamics and pathophysiology of aneurysms has been performed and there is an improved understanding of the associated complications. We present an extensive review of the recent literature highlighting the major advances in the field.