Purpose of reviewTo discuss in detail the efficacy and safety of rituximab (RTX) for induction and maintenance of remission in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) who are either treatment naive, relapsing or refractory to standard of care.Recent findingsIn treatment naive AAV patients, RTX without maintenance treatment is as effective as cyclophosphamide (CYC) followed by azathioprine (AZA) for maintenance for up to 18 months. RTX is superior to CYC for induction of remission in patients with relapsing AAV. Nevertheless, long-term follow-up shows relapsing disease in up to 50% of patients with proteinase 3-ANCA, irrespective of the induction regimen. RTX is useful in patients with refractory AAV, but percentages of patients achieving complete remission differ between series. RTX seems more effective than AZA for maintaining remission, but detailed results from prospective studies are being awaited. Adverse events do not differ between RTX and classical induction regimens, but infections related to hypogammaglobulinemia and neutropenia could be items of concern with repeated administration of RTX.SummaryRTX is an alternative for CYC for induction of remission in generalized AAV and could be first choice for relapsing patients and patients refractory to CYC. RTX is promising for maintenance of remission, but long-term safety should be awaited.