Rheumatoid vasculitis (RV) is one of the most severe extra-articular manifestations of rheumatoid arthritis, with significant morbidity and mortality, requiring aggressive treatment with corticosteroids and/or immunosuppressants. Recently, biological drugs were included in its therapeutic armamentarium. The objective of this study was to perform a systematic review on the use of biological drugs in the treatment of RV. A systematic literature review was performed based on PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations and searching articles in MEDLINE/PubMed, Cochrane, SciELO, Scopus, and Virtual Health Library electronic databases. Secondary references were also evaluated. The methodological quality of the selected studies was evaluated by the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) criteria. Altogether, five articles, assessing the use of biological drugs, were included. Globally, 35 patients participated in the studies, of which 21 were treated with rituximab (RTX) in cycles of 1000 mg every 2 weeks; 9 used infliximab 5 mg/kg; 3 used infliximab 3 mg/kg; and 2 used etanercept 25 mg twice/week. In general, an improvement in clinical picture, reduction of the mean daily dose of corticosteroids, and improvement in the Birmingham Vasculitis Activity Score was achieved by the end of the treatment. Complete remission occurred in almost 70% of the cases. The adverse effect rate was 34%, mainly due to infections. There were two deaths, one due to sepsis and the other due to uncontrolled vasculitis, after the biological drug withdrawal, following the development of sepsis. Based on the results of the present review, we believe that the use of biological therapy such as RTX and anti-tumor necrosis factor α can be beneficial in treating this complication.