Coronary artery aneurysm (CAA) is an uncommon coronary disease, with a reported incidence in adults ranging from 0.33 to 4.9%.It is usually considered a variant of coronary artery disease (CAD). CAA is associated with thrombus formation due to abnormal laminar flow, as well as abnormal platelet and endothelial-derived pathophysiologic factors within the CAA. CAA identified in the context of acute coronary syndrome (ACS) poses several unique management challenges. Optimal antiplatelet and anticoagulant therapy is the mainstay of therapy. Percutaneous intervention for CAA is associated with complications including distal embolization of thrombus, no-reflow phenomenon, stent malposition, dissection, and rupture. There are currently no accepted guidelines to direct the management of CAA in patients presenting with ACS. Preference for conservative vs. surgical or catheter-based management is controversial. We review the literature and report different treatment strategies for two cases with both CAA and ACS.