Abstract <p>IgA nephropathy (IgAN) is the most common primary glomerulonephritis, typically presenting early in life, often in young adults but also frequently in childhood. This chronic disease can account for up to 50% of cases progressing to kidney failure, particularly when it clinically begins at a young age. Currently validated treatments, such as renin–angiotensin blockers, SGLT-2 inhibitors, and corticosteroids, can slow disease progression, but with limited efficacy. In light of this, novel therapies targeting specific pathophysiological pathways are being developed, with increasing evidence supporting their effectiveness in reducing proteinuria and stabilizing glomerular filtration rate (GFR) in IgAN. In this review, we aim to highlight the main pathological pathways potentially targeted by these therapies and then present the most advanced treatments under development for IgAN. The key targeted pathways are the gut mucosal GdIgA1 synthesis and the lectin and alternative pathways’ activation of the complement system, through B cell depletion or modulation and inhibition of complement proteins. We also discuss the potential role of these treatments in pediatric patients.</p> Graphical Abstract <p></p>