A 90-year-old female had developed intractable otitis media 10 years previously. At the time, based on the findings of positive antineutrophil cytoplasmic antibody (ANCA) in her sera, the middle ear involvement was considered to be due to ANCA-associated vasculitis, and oral prednisolone was started. During the tapering period, she developed facial nerve palsy and was treated with methylprednisolone pulse therapy. Thereafter, the oral prednisolone was gradually tapered and finally stopped 3 years ago. However, she developed swelling of the lower leg, and multiple small ulcers emerged, which worsened 3 months previously. On physical examination, multiple, geographic ulcers and bloody vesicles were observed within a purpuric patch on the anterior aspect of her left lower leg, and her serum myeloperoxidase-ANCA was elevated (>134 U/mL). Biopsy specimen revealed diffuse granulomatous inflammation with lymphocyte and neutrophil infiltration, histiocytes with giant cells, red blood cell extravasation, and secondary small vessel vascular impairment with fibrinoid changes. There was no lung or renal involvement. We diagnosed the case as limited granulomatosis with polyangiitis, and reviewed MPO-ANCA positive GPA cases.