An 80-year-old woman, who had been received steroid therapy to treat diffuse alveolar hemorrhage from July, 2007, was admitted because of fever, eye pain and exophthalmos on 23 July, 2008. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was positive, but pulmonary involvement did not recur. 67Ga scintigraphy revealed intense uptake in bilateral orbital walls and Gd-enhanced MRI indicated orbital inflammatory pseudotumor and hypertrophic pachymeningitis. Although proteinase 3 (PR3)-ANCA was negative, we diagnosed Wegener's granulomatosis (WG) based on the following ELK criteria: upper airways (E); saddle nose, chronic otitis media and exophthalmos, lungs (L); alveolar hemorrhage, and kidneys (K); occult-blood and protein positive urine. Combination therapy with increased doses of prednisolone and cyclophosphamide improved her symptoms satisfactorily. We report a rare case of WG accompanied by orbital inflammatory pseudotumor without exacerbation of sinonasal involvement.