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Aortic diseases have high mortality and are usually late or misdiagnosed. Especially in patients with inflammatory vasculitis, diagnosis is often confused with other causes of chest pain and this causes a delay in diagnosis. Vascular complications are the most important predictors of mortality and morbidity in Behcet’s disease and also polyarteritis nodosa. The diagnosis of aortitis is usually obtained by vascular imaging, but partly made only by biopsy on occasion of an operation. Here we present a case of pathologically proven fatal aortitis and aortic dissection in a patient with Polyarteritis nodosa and Behcet's disease.