Ocular signs and symptoms may be the initial signs of systemic or limited Wegener's granulomatosis. The diagnosis is difficult to make on ophthalmic manifestations alone, because clinical and histo-pathological markers are not specific enough. By means of the cANCA serum test it is possible to make the diagnosis in an early stage and to prevent more disastrous complications. The relation of cANCA titre and the clinical and histopathological findings of five patients with an acute or chronic Wegener's granulomatosis are discussed.