Objetives: Immunoglobulin A vasculitis (IgAV) is an inflammatory disease with a controversial treatment based in corticosteroids as first line. To review cases of patients with IgA vasculitis (IgAV) treated with rituximab (RTX) and assess disease characteristics, treatment efficacy and safety.Methods: We conducted a systematic literature review according to PRISMA guidelines. We searched Pubmed, Web of Science, Embase and Scopus, selecting articles with information on IgAV and RTX treatment up to February 2019, with no language limitations. We extracted data on patient characteristics, disease evolution, treatment safety and efficacy. We created a database and analyzed it using statistical software package SPSS v 22.0.Results: We extracted clinical data for 43 IgAV patients treated with RTX. Distribution by sex was similar, and the median age at diagnosis was 16 (range 2 months to 70 years). The majority of patients were diagnosed at a pediatric age (24 patients under 18, 55.81%). The time of disease evolution until RTX administration greatly varied. An important number of patients suffered renal complications (86%) before RTX treatment.The frequency of adverse effects from RTX was low (7%, hypersensitivity with no treatment interruption). In terms of disease evolution, 41 patients (95.3%) presented clinical improvement, 15 patients had recurrence after initial remission (34.9%) and 34 patients (79.1%) achieved full remission after completing treatment. None of the patients treated with RTX and included in our systematic review died.Conclusions: RTX is efficacious in patients with IgAV. A high percentage of patients achieved complete remission with a favorable safety profile.PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.