Immunoglobulin A nephropathy is a primary glomerulopathy, with prevalence ranking highest in the Pacific region, followed closely by Europe, but rare in Africa. Although practice patterns likely have contributed to its prevalence, there are genetic and environmental factors that contribute as well. Management has evolved over the past decade, with recent rapid advances in diagnosis, prognosis, and therapies. This review summarizes the history, pathogenesis, and diagnosis of immunoglobulin A nephropathy and also cites relevant clinical trials, latest treatment options, and unanswered questions.