INTRODUCTION: Information on pregnancy outcomes in Anti Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis is limited to small case series. We systematically reviewed the literature to determine pregnancy outcomes in women with ANCA-associated vasculitis, to help with counselling and management of these women. METHODS: We searched Medline, Embase, Web of Science and PubMed from inception until July 2017, using keywords and subject headings related to pregnancy and ANCA-associated vasculitis. Abstract screening, data extraction and risk of bias assessment using the National Institute of Health tool for case reports and case series were performed in duplicate. Vasculitis- and pregnancy-related outcomes were obtained, and pooled incidence (95% confidence intervals) calculated. RESULTS: After screening 628 titles and 183 full texts, we included nine studies reporting 124 pregnancies in 89 women. Most studies (77.8%) had low risk of bias. There were 119 (91%) live births with a mean gestational age of 38.24 (37.35, 39.14) weeks and birth weight of 3.16 (2.95, 3.38) kg. Vasculitis flares were mostly in the ear, nose and throat 12.7% (4.9, 20.6%) and lungs 8.9% (2.9, 14.8%). Most patients were on glucocorticoids (43.5%) and/or Azathioprine (24.2%), increased doses of which successfully treated flares. Adverse outcomes included prematurity 10.3% (3.5, 17.1%), preeclampsia 5.5% (1.4, 9.5%), fetal growth restriction 4.9% (0.9, 8.9%) and congenital anomalies 3.7% (0.3, 7%). CONCLUSION: Women with ANCA-associated vasculitis can be assured that despite large numbers of flares during pregnancy, severe flares are rare and easily managed by high-dose glucocorticoids and immunosuppressants. Pregnancy outcomes are comparable to low-risk populations, apart from an increased risk of preterm birth.