The best treatment of IgA nephropathy (Berger's disease) is not well defined and at present no causal therapy is available. Although initially considered benign, we now recognize it as a common cause of end-stage renal disease and the natural history of IgA nephropathy is quite variable. Standard care includes angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers (ARBs) and corticosteroids, in some cases combined with immunosuppressive drugs. The antiproteinuric and renoprotective effects of ACEIs and ARBs in IgA nephropathy have been firmly established. Treatment with corticosteroids is effective in reducing proteinuria and renal injury. The addition of cytotoxic immunosuppressive agents (cyclophosphamide and azathioprine) can be of benefit in patients with a rapidly progressive disease course. Little information is available about the clinical efficacy of tonsillectomy on long-term renal survival in patiens with IgA nephropathy; at present it cannot be recommended. The treatment of the disease is a work in progress; only better knowledge of its pathogenesis will eventually offer novel therapeutic approaches.