The authors present a case of Wegener’s granulomatosis that had as first symptom scleritis, uveitis and proptosis. Because of rapidly progressive glomerulonephritis, the patient was admitted three months later. On the 12 day, third of cyclosphosphamide therapy, the patient died in respiratory distress. Autopsy disclosed focal and segmental necrotizing glomerulonephritis with crescent formation, and medium and small vessels necrotizing vasculitis in the ling, liver, and spleen. Necrotizing granulomas, massive alveolar hemorrhage, capillaritis, and extensive fibrosis in the lung was also observed. Ocular, lung and kidney involvement in the limited and diffuse forms of Wegener’s granulomatosis are discussed. A statement on ANCA’s value in the diagnosis and follow-up of Wegener’s patients is also made.