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A 31-year-old male who presented with a true aneurysm of the ascending aorta had for 5 years been seropositive for HIV-1 following intravenous drug abuse. Elective aneurysmectomy was refused. Controls by computerized tomography and echocardiography gave evidence of a progressive dilatation during the following 8 months. In February 1992 a further increase of the aneurysm with severe thoracic pain necessitated an emergency graft implantation. Histopathology of the resected aorta revealed a granulomatous giant cell mesaortitis. The postoperative course was uneventful and the patient remained free of cardiovascular symptoms, but died 25 months later due to multiple HIV-associated opportunistic infections. The differential diagnosis (Marfan's syndrome, vasculitis due to tuberculosis, syphilis and other infectious agents, rheumatological diseases, HIV-associated vasculitis) and the etiopathological considerations are discussed.