Abstract: Objective To explore the clinical manifestations of myeloperoxidase-antineutrophil cytoplasmic antibody (MPOANCA) associated hypertrophic pachymeningitis. Methods One patient with MPO-ANCA associated hypertrophic pachymeningitis was treated in Tongji Hospital, Huazhong University of Science and Technology.The clinical characteristics and treatment were retrospectively analyzed and the related literatures were reviewed. Results The patient was a 56-year-old woman complaining of intermittent headache, fever and myalgia in both legs, along with positive MPO-ANCA.Magnetic resonance imaging (MRI) showed diffuse thickening of the dura mater and ischemia in the alba.Cerebral magnetic resonance angiography revealed that blood flow was cut off at the origin of the right median cerebral artery and stenosis was found at the proximal of both anterior cerebral arteries.The symptoms were improved with intravenousglucocorticoid and cyclophosphamide treatment.She was diagnosed with mild renal dysfunction and interstitial pneumonia during follow-up.MPO-ANCA associated vasculitis was considered as one of the major causes for hypertrophic pachymeningitis.In this article, we reviewed the literatures published about MPO-ANCA associated hypertrophic pachymeningitis and found out that the ratio of patients with MPO-ANCA associated hypertrophic pachymeningitis combined cerebral vascular, renal and pulmonary involvement was relatively high. Conclusion MPO-ANCA associated hypertrophic pachymeningitis is a rare disorder, which occurs mostly in elderly female patients with headache as the initial symptom.This rare disorder and its involvement of other organs or systems require more attention from clinical doctors.