The immunopathologic disease, Wegener's granulomatosis, presents a challenge to the anesthesiologist due to multisystem involvement resulting in potential abnormalities of the airway, respiratory, circulatory, renal, and central/peripheral nervous systems. It is a systemic vasculitis of small, medium and occasional large arterial involvement. A familiarity with the proper approach to perioperative management is essential. Additional considerations must be made as problems arise from immunosuppressant and corticosteroid treatment.