Drug-induced vasculitis is an inflammatory vasculopathy associated with drugs of almost every class and accounting for approximately 3% of the vasculitides. Although small vessel disease limited to the skin is the most common form, involvement of blood vessels in virtually every organ system may occur. Given its similarity to other vasculitides and the absence of reliable confirmatory tests, it remains a diagnosis of exclusion. Cell-mediated and humoral immunity invariably play an important role in pathogenesis. To date, the most likely mechanism is related to the development of antineutrophil cytoplasmic autoantibodies. Withdrawal of the suspected causative drug is essential, but corticosteroids, immunosuppressive agents or, rarely, plasmapheresis may be necessary in multiorgan or life-threatening disease. The mortality is up to 10%.