To provide an up-to-date and comprehensive review of Wegener's granulomatosis (WG) as a disease entity, focusing on the ophthalmic manifestations and management options. A search of Medline was undertaken between 1966 and 2005 regarding WG, systemic vasculitis, and the ocular manifestations of WG. Major ophthalmic and medical textbooks also were reviewed for content, as well as original references. Involvement of ocular and orbital structures in patients with WG is common and may be a presenting feature. The ocular manifestations range from mild conjunctivitis and episcleritis to more severe inflammation with keratitis, scleritis, uveitis, and retinal vasculitis. Involvement of the nasolacrimal system and orbital tissues also can occur. Except for some cases of anterior segment inflammation, the ocular involvement will not respond to topical agents, but rather to systemic antiinflammatory and immunosuppressive regimens. Surgical intervention may be of value for obtaining tissue diagnosis, in achieving orbital decompression in cases of significant orbital disease with optic nerve compromise, or in cases of nasolacrimal duct obstruction. WG is an important clinical entity that needs to be recognized early and treated appropriately. Ophthalmic manifestations are frequently encountered and can result in significant morbidity and even blindness. The management is challenging and often requires a multidisciplinary approach.