Rhabdomyosarcoma (RMS) is an exceedingly rare tumor in the renal pelvis. We report a 76-year-old female patient with RMS presenting with a mass in the right renal pelvis on a series of imaging studies that simulated transitional cell carcinoma. To our knowledge, this is the second reported case of this entity and is the first description of its imaging findings. Here, the differential diagnoses of the imaging findings in our case are discussed and the literature is reviewed.