The most common primary glomerulonephritis in the world is Immunoglobulin A nephropathy (IgAN). Nevertheless, the pathogenesis of IgAN has not yet been identified. The histological diagnosis requires either dominant or co-dominant IgA staining in renal biopsy. IgAN patients could present with various clinical manifestations, ranging from asymptomatic abnormalities observed on urinalysis to rapidly progressive glomerulonephritis. Since each patient shows a significantly different disease course, the disease progression risk calculator may help the patients discuss treatment strategies with doctors and understand prognosis. Supportive treatment, including angiotensin-converting enzyme inhibitor or angiotensin II receptor blocker, is essential for initial treatment of IgAN. The role of various immunosuppressants currently available in IgAN is still controversial. In the case of corticosteroids, proteinuria is reduced, but associated with an increased risk of various complications, including infection, in long-term use. Although its effectiveness has not yet been fully proven, clinical trials of various novel drugs have recently been conducted. In this presentation, an overview of IgAN and its latest treatment options will be reviewed.