Background <p>Idiopathic nephrotic syndrome (INS) is the leading glomerular disease in children, often responding well to steroids. However, frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) require the introduction of steroid-sparing agents to mitigate steroid toxicity.</p> Methods <p>This retrospective, pilot study including 6 patients with FRNS or SDNS assessed the efficacy and safety of obinutuzumab as a first line of anti-CD20 therapy.</p> Results <p>Using one dose of obinutuzumab, complete remission was achieved in all 6 patients within 11&#xa0;months. Remission rate was 100% up to 12&#xa0;months post-injection, and 80% at both 24 and 56&#xa0;months. Infusion-related reactions occurred in 83% of the patients, primarily mild and transient; one patient required immunoglobulin supplementation but no severe infections were noted.</p> Conclusions <p>These preliminary results suggest that obinutuzumab is a promising first-line anti-CD20 therapy for FRNS and SDNS in children, offering sustained remission with a good safety profile.</p> Graphical Abstract <p>A higher resolution version of the Graphical abstract is available as Supplementary information</p> <p></p>