Aortic dissection is accompanied by fever in about one third of the patients. However, fever of unknown origin as the predominant manifestation of aortic dissection seems to be extremely rare. A review of the English literature revealed only 3 patients characterized by fever as the principal sign of aortic dissection. Herein an additional patient is reported. All 4 patients presented with pain or discomfort in the chest, back or abdomen, followed by persistent fever, lasting 5-11 weeks and associated with anemia and a high sedimentation rate. The outcome was favorable in all cases regardless of the location of the dissection or the type of treatment.