Acute aortic diseases represent a group of complex severe and often fatal medical conditions. Although they are significantly rarer than cardiac or thromboembolic events, they are an important differential diagnosis to be ruled out, e.g., in the clinical work-up of acute chest pain. Treatment, especially surgical interventions, depends on the progression, extent and size of the pathology and whenever possible should be performed in specialized centers with the appropriate experience. Intensive care monitoring is advisable as a range of peracute complications can occur even in initially stable patients. Depending on the clinical presentation and affected structures, a number of severe complications need to be anticipated by critical care physicians. Additionally, a notable symptom is severe and refractory hypertension, especially in the acute phase. This article provides a summary of the most frequent clinical pictures and corresponding treatment options. Furthermore, the principles of initial patient stabilization and treatment as well as the perioperative management of complex surgical procedures on the aorta are discussed.