Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), particularly myeloperoxidase (MPO)-ANCA-positive granulomatosis with polyangiitis (GPA), rarely present with acute arterial digital ischemia or necrosis.<i> </i> We report a 68-year-old woman with a history of seropositive rheumatoid arthritis, suspected connective tissue disease (CTD)-associated interstitial lung disease, scleromalacia perforans, and osteoporosis who presented with acute ischemia of the left hallux that progressed to involve multiple digits despite therapeutic anticoagulation. The gene discussed is MPO; the disease is rheumatoid arthritis.