The term acute aortic syndrome refers to a heterogeneous group of conditions that cause a common set of signs and symptoms, the foremost of which is aortic pain. Various pathologic entities may give rise to this syndrome, but the topic has come to focus on penetrating aortic ulcer and intramural hematoma and their relation to aortic dissection. Penetrating aortic ulcer is a focal atherosclerotic plaque that corrodes a variable depth through the intima into the media. Intramural hematoma is a blood collection within the aortic wall not freely communicating with the aortic lumen, with restricted flow. It may represent a subcategory of aortic dissection that manifests different behavior by virtue of limited flow in the false lumen. This article reviews the current literature regarding acute aortic syndrome, focusing on management options.