Agnogenic myeloid metaplasia (AMM), a myeloproliferative disorder of c
lonal origin, can affect the kidneys via several mechanisms. Associate
d disorders include asymptomatic interstitial extramedullary hematopoi
esis, obstructive uropathy and nephrolithiasis. Currently, there are n
o descriptions of the nephrotic syndrome associated with AMM. We prese
nt a case of AMM associated with the nephrotic syndrome, a mesangial p
roliferative glomerular lesion and extramedullary hematopoiesis.