Patients with granulomatosis with polyangiitis occasionally present with cutaneous manifestations, which are important clues for the early diagnosis. Although pyoderma gangrenosum-like ulcers are rarely observed, a unique case with unusual clinical features is presented herein. A 75-year-old woman with positive proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) repeatedly developed aseptic abscesses on the abdomen, buttock, lower legs, and forearms. Histopathological features of biopsy taken from ulcers showed necrotizing granulomatous inflammation with multinucleated giant cells without leukocytoclastic vasculitis. The present case was initially diagnosed as limited granulomatosis with polyangiitis without renal and lung involvement. However, two years later, she died of cerebral hemorrhage.