As a chronic, focal, necrotizing granulomatous angiitis Wegener's granulomatosis mainly affects the upper respiratory tract, the lungs and kidneys. Various other organs may be affected including te eyes. Ocular manifestation of Wegener's granulomatosis may result from granulomatous sinusitis that has proceeded into the orbit causing nasolacrimal duct obstruction and proptosis, and affecting eye muscles as well as the optic nerve. Another possible picture is presented by focal vasculitis in the form of conjunctivitis, episcleritis, scleritis, uveitis and granulomatous vasculitis of the retina and optic nerve. In 13 patients out of 26 examined the eyes were found to be involved. The etiology of Wegener's granulomatosis is unknown. The prognosis of this generally lethal disease could definitely be improved by early treatment with cyclophosphamide in combination with glucocorticosteroids.