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Wegener's granulomatosis (WG) is a chronic, focal, necrotizing granulomatous angiitis, which mainly affects the upper respiratory tract, the lungs and the kidneys. We present the case of a 26-year-old female patient complaining of pain and increasing swelling of the upper right eyelid with exophthalmos and diplopia as the first manifestation of a classic WG. Computer tomography revealed a homogeneous tumor arising from the right lacrimal gland. Chest x-ray revealed a tumor of the right lung. Microhematuria disclosed a slight renal involvement. Positive ANCA titer and C-ANCA anti-PR3, as well as a positive lung biopsy, confirmed the diagnosis of WG. Treatment with prednisone and cyclophosphamide was initiated. One year later, the patient's condition was good, and the ophthalmological examination was normal. WG should be considered in the differential diagnosis of an orbital tumor. In some rare cases, orbital disease can be the first clinical manifestation of the classic form of WG.