We report on a cese of a 36-year-old female patient with the chief complaint of right loin pain for 1 week. The image studies with ultrasound, computed tomography, and angiography all revealed a right renal mass. The pathological finding was a primitive neuroectodermal tumor (PNET) of the right kidney. The patient was well without recurrence or metastatic disease 1year after surgery. We report on this case and review the literature.