A 71-year-old man developed protracted nausea, a 6.75-kg weight loss, acute renal failure, and pulmonary hemorrhage. He had positive results on a perinuclear antineutrophil cytoplasmic autoantibody assay and an antimyeloperoxidase enzyme-linked immunosorbent assay (level = 130.8 U; positive >20.0 U) and negative results on an antiproteinase 3 enzyme-linked immunosorbent assay (level = 3.9 U; positive >20.0 U). Although he did not have the typical pattern of antineutrophil cytoplasmic antibodies with cytoplasmic staining, his findings were considered most compatible with WG. His renal failure did not resolve with hemodialysis, high-dose methylprednisolone sodium succinate, cyclophosphamide, and plasmapheresis. He had progressively decreasing strength, mental status, and ability to tolerate tube feeding and died approximately 5 months after his initial symptom of nausea developed. A complete autopsy confirmed the diagnosis of WG with necrotizing granulomatous and fibrinous vasculitis with neutrophils and karyorrhectic debris involving the kidneys, testes, appendix, liver, spleen, lungs, pancreas, lymph nodes, small and large intestines, trachea, aorta, pericardium, myocardium, and both orbits (listed in order of decreasing histological severity).