We describe the first case, to our knowledge, of the concordant presentation of nephrotic syndrome in twins with simultaneous mutations in <i>PAX2</i> and <i>MYO1E</i>.<h4>Case report</h4>At 32 months and 33 months of age, monochorionic, diamniotic twin girls presented with nephrotic syndrome. The gene discussed is PAX2; the disease is nephrotic syndrome.