Immunoglobulin A (IgA) nephropathy is the most common form of primary glomerulonephritis globally and a frequent cause of severe kidney failure in young adults. Kidney biopsy is used to diagnose the condition. Despite it being identified more than fifty years ago, understanding of the disease's pathophysiology is limited, and there is currently no targeted treatment apart from supportive therapy. A number of clinical drug trials are now being undertaken, giving hope that effective treatment may become available in a few years. The patient care pathway usually starts in general practice, but assessment by a nephrologist is necessary for further diagnostics. In this clinical review article, we will describe the investigation, treatment and prognostic assessments for IgA nephropathy.