A 46-year-old man was referred for evaluation of a 13-cm renal mass. Right radical nephrectomy was performed. Pathologic evaluation revealed inflammatory myofibroblastic tumor (IMT) and renal cell carcinoma. To our knowledge, only 28 cases of IMT associated with the kidney have been reported in published studies. This is the first reported case of IMT associated with malignancy in the urogenital tract. Some investigators have suggested that biopsy-proven IMT can be managed conservatively, but the findings of this report have demonstrated the need for radical nephrectomy in this patient population to rule out associated malignancy.