Wegener's granulomatosis is a granulomatous necrotizing vasculitis characterized by its predilection to affect the upper and lower respiratory tracts and kidneys. Ocular manifestations such as keratitis, conjunctivitis, scleritis, episcleritis, nasolacrimal duct obstruction, uveitis, retroorbital pseudotumor with proptosis retinal vessel occlusion, and optic neuritis have all been described. We present a case of limited Wegener's granulomatosis presenting with proptosis. A 57 year old woman with a history of Graves' disease 20 yrs ago, presented to her ophthalmologist with new onset of exophthalmos of right eye. An MRI of the brain and orbits revealed a soft tissue mass behind the right orbit. The biopsy of the mass revealed transmural inflammation with fibrinoid necrosis consistent with necrotizing vasculitis. She was diagnosed with Wegener's granulomatosis limited to the eye and was treated with oral cyclophosphamide and prednisone followed by weekly methotrexate with good response. Though ocular manifestations of Wegener's granulomatosis are well described, a review of the literature revealed that exophthalmos as the lone presenting manifestation is quite rare. Only two cases have been reported in the English literature since 1977. This case illustrates the importance of considering a diagnosis of limited Wegener's granulomatosis presenting with proptosis of the orbit.