A 59-year-old woman was admitted with scleritis, sinusitis, skin eruptions, nodular lesions of both lung fields in chest X-ray films and renal failure. Skin biopsy and elevation of the titer of anti-neutrophil cytoplasmic antibody confirmed Wegener's granulomatosis. A right nodular goiter was palpated and a diagnosis of thyroid cancer was made based on aspiration cytology. Although combined therapy with cyclophosphamide and corticosteroid was started and the Wegener's granulomatosis improved and disappeared except for the renal lesion, the renal failure worsened and she died. Apparently only 2 cases of Wegener's granulomatosis complicated with carcinoma as in this case have been reported.