Several types of human immunodeficiency virus (HIV)-associated vasculitides, including polyarteritis nodosa-like syndromes have been reported in patients with HIV infection. The pathogenic mechanism is thought to be related to either circulating immune complexes or direct endothelial involvement by HIV per se. In HIV-related necrotizing systemic vasculitis, affected arteries are smaller than those seen in classic polyarteritis nodosa, and it rarely becomes a multisystem and life-threatening disease with the exception of cases with heart, kidney, central nervous system, or gut involvement. We describe a case of systemic necrotizing vasculitis in an HIV-infected individual presented with fever, purpuric rash, and polyarthritis who eventually had development of mesentery artery thrombosis and nephrotic syndrome. The patient, solely under triple antiretroviral therapy, remains symptom free, with no evidence of his multisystem disease and no progression of his HIV infection after 10 years of follow-up. All patients with a vasculitis of unidentified etiology should be evaluated for HIV infection.