Immunoglobulin A nephropathy is now recognized as the glomerular disease most often associated with progressive renal failure in patients around the world. In many cases it is not known when the disease starts to inflict glomerular injury, but recent studies that have shown genetically determined abnormalities in glycosylation of the IgA molecule suggest that this may begin in early life. This review focuses on recent advances in our understanding of IgA nephropathy, with special emphasis on clinical aspects of the disease when it presents in children and adolescents. In addition, the sections dealing with therapeutic options for patients with IgA nephropathy concentrate on studies that have been carried out on children. Whenever possible, data from randomized controlled clinical trials have formed the basis for recommendations. Unfortunately, this is not always possible, because of the lack of such trials in patients with IgA nephropathy.