Spontaneous abdominal aortic dissection is a rare entity, often with a clinically unspecific presentation. The cause of the dissection is unclear. Angiography used to be the definitive diagnostic study, but today a correct diagnosis can be achieved with CT scanning and magnetic resonance angiography. The optimal form of management for the individual patient is not clearly established. Chronic dissections may best be managed conservatively, with close follow-up achieved with CT scanning and magnetic resonance. Acute and complicated dissections should be treated surgically with aortic and aortic branch replacement if it can be offered with low morbidity and mortality. In selected cases, resection of the ischemic organs may represent an alternative.