In recent years, with the improvement and popularization of anti-neutrophil cytoplasmic antibody (ANCA) detection technology, more and more patients with immunoglobulin A nephropathy (IgAN) have been tested positive for serum ANCA. The clinical value of ANCA is still unclear, and there is a lack of consensus on diagnosis and treatment strategies. This article reviews the clinical and pathological characteristics, diagnosis, and treatment features of IgAN patients with serum ANCA positivity through literature reading and analysis, aiming to provide a reference for standardized diagnosis and individualized management of this type of patient.