CASE REPORT Patient who consults for a recidivant orbital mass. Preliminary clinical and image studies suggested an initial differential diagnosis between a lachrymal gland tumor or an orbital located pseudotumor. However, the clinical evolution of the case required a re-evaluation of the preliminary diagnosis. The clinical, histologic and serologic findings were key in the final diagnosis. DISCUSSION Wegener's granulomatosis is a necrotizing vasculitis. Classically it affects the upper airways, lungs and kidneys. Between 30% and 70% of patients present ophthalmic symptoms during the course of the illness. Ophthalmic affectation prior to systemic manifestations is less frequent and could delay final diagnosis.