Agnogenic myeloid metaplasia (AMM), a myeloproliferative disorder of clonal origin, can affect the kidneys via several mechanisms. Associated disorders include asymptomatic interstitial extramedullary hematopoiesis, obstructive uropathy and nephrolithiasis. Currently, there are no descriptions of the nephrotic syndrome associated with AMM. We present a case of AMM associated with the nephrotic syndrome, a mesangial proliferative glomerular lesion and extramedullary hematopoiesis.