Microscopic polyangiitis (MPA), Wegener's granulomatosis and Churg-Strauss syndrome are known as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Cerebrovascular diseases have been rarely reported to be associated with MPA. In this study, we reported a 72-year-old man with MPA showing an initial presentation of pontine infarction, mononeuropathy multiplex and progressively deteriorating renal function. He was diagnosed with MPA on the basis of increased myeloperoxidase-specific ANCA activity, mononeuritis multiplex and the findings of renal biopsy. After receiving an aggressive treatment consisting of plasma exchange, his neurological deficit dramatically improved. For stroke patients with acute nephritis, the possibility of ANCA vasculitis should be considered. Early diagnosis may improve the prognosis.