Congenital malformations, tumors and aortic infections are rare and mostly asymptomatic. Unspecific clinical symptoms may cause delayed verification of the underlying disease. Contrast enhanced computer tomography- and magnetic resonance angiography are important sectional imaging methods for diagnostic completion. Consistent guidelines concerning diagnosis and therapy of rare aortic diseases are non-existent. Aortic tumors must be resected by open surgery, aortic infections in general require medical treatment and anomalies, if indicated, are treated more and more by endovascular or hybrid procedures. Therefore, it is recommended to treat these entities in an interdisciplinary approach in specialized aortic centers.