<p>Despite an increasing number of therapeutic options for pediatric patients with steroid-resistant nephrotic syndrome (SRNS), treatment resistance and risk of progression to kidney failure are still high. Children with monogenic forms of SRNS and resistance to non-steroidal immunosuppressants are at the highest risk. Advances in the understanding of SRNS pathogenesis have enabled the development of novel therapies that target genetic, immunologic, and metabolic mechanisms of disease development and progression. In this review, we summarize select contemporary cohort study findings, novel therapeutic agents and their mechanistic targets, and eight recent and ongoing clinical trials for pediatric patients with SRNS.</p> Graphical abstract <p></p>