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Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. The majority of cases have ocular, sinus and ear symptomatology. A case is presented with severe granulomatous disease of the sinuses with contiguous spread to the orbit producing exophthalmos and decreased vision. An orbital decompression relieved the pressure and preserved vision. The definitive medical therapy for WG is cyclophosphamide. Emergency orbital decompression may be necessary to preserve vision in WG in patients with rapidly decreasing vision secondary to high intraocular pressure produced by the granulomatous process.