IgA nephropathy affects almost 1% of the population and yet the diagnosis is often missed. This significant kidney disease is often progressive with 25% of the patients going on to end-stage kidney disease over the course of 25 years. This minireview describes the clinical presentations in children and young adults. Therapeutic options are discussed including angiotensin-converting enzyme blockade, steroids, cytotoxics, tonsillectomy, fish oil, vitamin E, singly or in combination, in order to modulate the rate of progression.