Polyarteritis nodosa (PAN)-associated glomerulonephritis presenting with nephrotic syndrome is not common. We describe a 69-year-old hypertensive man in whom PAN coexisted with IgA nephropathy presenting with severe nephrotic syndrome, livedo reticularis, testicular pain, and microaneurysm of renal vessels. Extensive literature review of PAN-associaled glomerulonephrilis was summarized. PAN-associated IgA nephropathy should be considered in a patient having PAN with nephrotic syndrome. Steroid therapy is an effective therapy in cases where these 2 diseases coexisted.