An 81-year-old man was hospitalized because of fever and pain in the temporal region. Temporal artery biopsy revealed temporal arteritis; steroid therapy was started. Chest computed tomography and kidney biopsy revealed interstitial pneumonia and necrotizing crescentic glomerulonephritis, respectively. Because his myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) level was 215.0 U/mL, a diagnosis of microscopic polyangitis (MPA) was made. The patient was discharged after reduction of the steroid dose. However, his respiratory symptoms exacerbated, necessitating rehospitalization. He died 1 week later due to respiratory failure. MPA rarely involves the temporal artery. In the cases of large vessel lesions, ruling out MPA is important.