A 22-year-old female who had been suffering from Graves' disease developed p-ANCA positive necrotizing vasculitis. Her Graves' disease has been treated with propylthiouracil (PTI) for 6 years, which failed to improve serum TSH. She developed transient and recurrent polyarthralgia 4 years before. Because of microscopic hematuria and polyarthralgia, She visited our hospital last year. She had hypergammaglobulinemia and microhematuria at that time. But no autoantibody was noted except anti-microsomal antibody at that time. Skin biopsy from her finger subdermal nodule revealed necrotizing vasculitis of small vessels. Serum p-ANCA was positive. She was admitted under the diagnosis of p-ANCA positive necrotizing vasculitis. Renal biopsy revealed mesangium proliferative glomerulonephritis. Since she developed telescoped sediments after admission, predonisolone (PSL) 50 mg daily was started. Clinical and laboratory findings improved quite well. PSL was gradually tapered after 6 weeks. She is being maintained in good condition on tapering doses of both PSL and PTI. The coincidence of Graves' disease and vasculitis is rare. This case may provide some important immunological insights into the study of pathogenesis of necrotizing vasculitis and Graves' disease.