The term "acute aortic syndrome" (AAS) refers to a spectrum of life-threatening thoracic aortic pathologies including intramural hematoma, penetrating atherosclerotic ulcer, and aortic dissection. Clinically, patients often present with characteristic aortic pain. AAS often leads to aortic rupture. Therefore, recognition of this condition, its prompt diagnosis, and timely treatment is crucial to obtain clinical success and improved overall survival. The management of AAS, however, remains a therapeutic challenge. Endovascular strategies have gained wide acceptance and now represent a minimally invasive alternative to traditional open surgery. Several studies have shown endovascular repair of varying thoracic aortic pathologies to be technically feasible with fewer complications than open surgery. In this review, the authors discuss AAS pathology and its management, with particular attention to the current role of endovascular aortic repair and its treatment.