Coronary artery aneurysms (CAA) are defined as a dilation of a coronary vessel greater than 1.5 times the diameter of a local reference vessel. While CAAs tend to be incidental findings on imaging, they result in complications, such as thrombosis, embolization, ischemia, arrhythmias, and heart failure. Among symptomatic cases, chest pain has been the most common manifestation of CAAs. This necessitates an understanding of CAAs as a cause of acute coronary syndrome (ACS) presentation. However, due to the unclear pathophysiology of CAAs and their variable presentation complicated by similar ACS conditions, there is no clear strategy for CAA management. In this article, we will discuss the contribution of CAAs to ACS presentations and review the current management options for CAAs.