Since the beginning of the HIV epidemic a wide variety of vascular inflammatory diseases have been described in HIV infected patients. In terms of the primary forms of systemic necrotizing vasculitis, there are no convincing data suggesting HIV infection increases the risk of development of any form of these diseases, but it is possible--though yet unproven--that HIV may lessen the chance of developing other forms (i.e. ANCA-associated disease and HBV-associated PAN). Secondary vasculitis resulting from unusual pathologic expressions of opportunistic infections has been reported and has important clinical significance. Finally, there does appear to be growing clinical, epidemiologic and pathologic evidence that several distinctive forms of vascular inflammatory disease occur in certain settings. These include aneurysmal disease of the large arteries of the brain occurring in children and a large vessel aneurysmal disease primarily affecting the aorta and its branches in young HIV-infected patients from sub-Saharan Africa. Further study of these disorders is necessary to identify specific epidemiologic features and pathogenesis.