Focal proliferative necrotizing IgA nephropathy is a type of glomerular disease, a group of diseases characterized by fibrinoid necrosis of glomerular capillary collaterals. Focal proliferative necrotizing IgA nephropathy belongs to the class of small-vessel vasculitis, in which thickening and breakage of glomerular capillary collaterals or fragmentation of the nuclei or even nucleolysis of the nuclei are observed under electron microscopy, often accompanied by the deposition of fibrillar-like material. Focal proliferative necrotizing IgA nephropathy is usually treated according to the patient’s clinical manifestations, pathologic typing and other comprehensive formulation of the treatment plan. It often manifests as simple microscopic hematuria, recurrent hematuria, proteinuria, nephrotic syndrome, acute renal failure, hypertension, etc. If the patient is not treated in time, it can progress rapidly, for example, to crescentic nephritis, or even to renal failure. Patients with focal proliferative necrotizing IgA nephropathy are recommended to go to regular hospitals in time and standardize the treatment under doctors’ guidance.