Background <p>Persistent proteinuria is an independent risk factor for poor prognosis in immunoglobulin A vasculitis nephritis (IgAVN). Recent studies have shown that telitacicept effectively reduces proteinuria levels in patients with IgA nephropathy (IgAN). The treatment has been associated with a significant decrease in galactose-deficient IgA1 (Gd-IgA1) levels. This study aims to elucidate the effectiveness and safety of telitacicept in treating refractory childhood IgAVN.</p> Methods <p>This is a single-center, retrospective observational study of seven children with IgAVN who had previously received glucocorticoids and at least one immunosuppressive therapy but still exhibited proteinuria. Telitacicept was administered subcutaneously once a week. Dosage was 80 mg for individuals weighing &lt; 40 kg and 160 mg for those weighing &gt; 40 kg.</p> Results <p>The study encompassed seven children, comprising two boys and five girls, with a median age of 15 years. Among the participants, six children (85.7%) exhibited a reduction in proteinuria, ranging from − 23.6 to − 97.5% compared to baseline levels. Conversely, one child (14.3%) did not show any decrease in proteinuria. Throughout the follow-up period, two children (28.6%) achieved complete remission, characterized by proteinuria level below 0.2 g/day/1.73 m² and negative dipstick test for blood. Additionally, all participants demonstrated a significant decline in urinary red blood cell counts. In terms of medication administration, all patients discontinued the use of steroids and immunosuppressants. Furthermore, there was a reduction in the incidence of hospitalizations. Importantly, no serious adverse reactions were reported.</p> Conclusions <p>Telitacicept has demonstrated both efficacy and safety in treating children with refractory IgAVN.</p> Graphical abstract <p></p>