140 patients with biopsy-proven Wegener's granulomatosis were examined during a 16-year period at the Mayo Clinic. Forty patients had diverse and explosive ophthalmic involvement, including conjunctivitis, episcleritis, scleritis, corneal ulceration, uveitis, retinal vasculitis, optic neuropathy, orbital mass, orbital cellulitis, and obstruction of the nasolacrimal duct. The multiple ophthalmic and systemic complications in these 40 patients and the importance of establishing the pathologic diagnosis are discussed. Treatment with corticosteroids and immunosuppressive agents has dramatically improved the prognosis, although substantial mortality (four patients died of Wegener's granulomatosis) and ocular morbidity (three eyes were enucleated) are still associated with this disease.