AbstractIgA nephropathy is the most-common primary glomerulonephritis worldwide, and about 20%50% of patients develop progressive renal failure. The pathogenesis is still unknown and treatment has not yet been established. Knowledge concerning childhood IgA nephropathy has expanded greatly in the last 10 years, and its importance as the major form of glomerulonephritis and major contributor to end-stage renal disease is also becoming apparent in children. This review focuses on the pathophysiology and treatment of IgA nephropathy in children.