This is a report of a patient who was diagnosed with acute promyelocytic leukemia (APL) last 2007 and was given a standard chemotherapeutic regimen of anthracycline, all-trans-retinoic acid and methotrexate. The patient completed treatment and recovered. Twelve years after chemotherapy, the patient was diagnosed to have renal tumor. The patient presented with intermittent episodes of non-bothersome flank pain. He was managed as a case of renal newgrowth, left, stage II (cT2bN0M0), which was eventually found to have intermediate grade, round cell sarcoma not further classified of the kidney. Immunohistochemical studies and literature review point to a newly classified subtype of sarcoma or a primitive neuroectodermal tumor, both of which are rarely found presenting in the kidneys.