The incidence of venous thromboembolism (VTE) in ANCA-associated vasculitis patients varies in different populations. Moreover, the risk factors for VTE in these patients are poorly described due to the small number of events. Ovid MEDLINE, EMBASE, and the Cochrane Library were searched for eligible articles. The inclusion criteria included observational studies that enrolled patients age ≥ 18 years diagnosed with ANCA-associated vasculitis. The incidence of VTE is the outcome of interest. Of 1362 citations, a total of 21 studies (n = 4422) dated from 2006 to 2019 were included in the systematic review. The mean age was 54.2 ± 4.0 years. Most were male (52.0%) and Caucasian (80.9%). With a mean follow-up duration of 5.2 ± 2.8 years, the pooled incidence of VTE in ANCA-associated vasculitis patients was 12.4% (95% CI, 8.8-17.2). Of these, 63.4% (95% CI, 57.3-69.1) had deep vein thrombosis and 26.3% (95% CI, 17.6-37.4) had pulmonary embolism. Recurrent VTE occurred in 10.0% (95% CI, 5.2-18.6). From the metaregression adjusted for age, sex, and ethnicity; positive MPO-ANCA, increasing Birmingham Vasculitis Activity Score at time of vasculitis diagnosis, and presence of renal involvement were positively associated with increased VTE events. Positive PR3-ANCA profile was inversely associated with increased VTE events. Increasing follow-up duration was not associated with increased VTE events. VTE in ANCA-associated vasculitis is common. Positive MPO-ANCA, increasing vasculitis activity, and presence of renal involvement were significant risk factors for VTE while positive PR3-ANCA was inversely associated with increased VTE. Key Points • Venous thromboembolism (VTE) is common in ANCA-associated vasculitis with a pooled incidence of 12.4% • Deep vein thrombosis accounts for two-third of total VTE cases • Positive MPO-ANCA profile, higher disease activity at ANCA-associated vasculitis diagnosis, and renal involvement are risk factors for VTE • Positive PR3-ANCA profile is protective factor for VTE.