A case of polycythemia vera (PV) associated with immunoglobulin A nephropathy (IgAN) in a 19-year-old woman is described. A literature search for PV associated with renal disease was also conducted. It has been reported that myeloproliferative neoplasms may play a role in the development of some glomerular diseases. The JAK2 mutation plays a role in the pathogenesis of myeloproliferative neoplasms and it may therefore also be responsible for this association.