Several related disorders comprise the spectrum of nonmuscle myosin heavy chain 9-associated (MYH9) nephropathy. The contribution of variants in this single MYH9 gene to ethnic differences in the incidence rates of end-stage renal disease is now clearly established. The importance of recognizing the role of MYH9 in these inherited kidney disorders goes beyond simple disease association; there may well be effects on clinical outcomes in patients on dialysis and after kidney transplantation. MYH9 polymorphisms may affect treatment outcomes in severe kidney disease and such gene effects are rarely encountered in practice.