Inflammatory myofibroblastic tumor (IMT) is an extremely rare disease composed of myofibroblast cells and inflammatory infiltrates. There are different sites of the urogenital system affected by IMT-bladder, prostate and kidney. We report a case of a 59-year-old male patient presented with abdominal pain, gross hematuria and a renal mass treated with partial nephrectomy. The final diagnosis was renal inflammatory myofibroblastic tumor. Despite recent improvements in imaging technology, preoperative diagnosis of IMT remains a dilemma. It is therefore mandatory to carry out clinical interpretation, careful histologic examination, and immunohistochemical studies which will generally determine the appropriate diagnosis and patient management.