Acute aortic syndrome (AAS) describes the acute presentation of patients with characteristic "aortic pain" caused by one of several life threatening thoracic aortic conditions including aortic dissection, intramural hematoma or penetrating atherosclerotic ulcer. Recent advances in imaging and therapeutic techniques have emphasized the importance of early diagnosis of acute aortic syndrome because it is crucial for survival. Not just because the cardiovascular community knows little, the management of AAS remains a therapeutic challenge, while diverse surgical and percutaneous strategies for the treatment of aortic syndromes are continuously evolving. As a result of increasing knowledge and better management strategies in this area, the outcomes of patients treated for acute aortic syndromes have improved. This review discusses the etiology and clinical presentation, but mainly focuses on modern management and both established and emerging therapeutic approaches to acute aortic syndromes.