Wegener?s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener?s granulomatosis with oral manifestation, which began as chronic mastitis.