Background <p>Recent investigations have indicated that telitacicept significantly diminishes proteinuria levels in adult patients diagnosed with IgA nephropathy (IgAN). The therapy has been correlated with a substantial reduction in galactose-deficient IgA1 (Gd-IgA1) levels. The present study endeavors to clarify the efficacy and safety profile of telitacicept in the treatment of IgAN among pediatric patients.</p> Methods <p>This study is a retrospective, observational, single-center research on 12 pediatric patients with IgAN treated with telitacicept as adjunctive therapy, mainly via weekly subcutaneous injections at 2.5&#xa0;mg/kg, up to a maximum of 160&#xa0;mg. During telitacicept treatment, ACEI use was maintained, and immunosuppressive doses were gradually reduced.</p> Results <p>A total of 12 patients with a mean age of 11.75&#xa0;years were enrolled. The median follow-up duration post-telitacicept initiation was 9&#xa0;months. The median 24&#xa0;h urinary proteinuria decreased from 0.98 (0.5, 9.8) g/1.73 m² at baseline to 0.36 (0.08, 2.49) g/1.73 m² at month 12. At the final follow-up, 7 patients (58.3%) achieved complete remission. During the follow-up period, routine urinalysis demonstrated a significant improvement in urinary red blood cell counts for all patients. No serious adverse events (AEs) were reported in any patient during treatment with telitacicept.</p> Conclusions <p>Telitacicept has exhibited both effectiveness and safety in the treatment of children diagnosed with IgAN.</p> Graphical abstract <p></p>