Periureteral varices are rare, and periureteral varices with accompanying pyelitis have been even more rarely reported. Our patient with flank pain was diagnosed with pyelitis because her CT scan showed thickening and enhancement of the ipsilateral renal pelvic wall and her clinical manifestations and laboratory results were well correlated with the diagnosis. Moreover, the author diagnosed periureteral varices because periureteral, tortuous, enhancing vascular structures were detected and 3-dimensional rendering technique of the CT scan showed that periureteral varices were connected to the ipsilateral renal vein. We experienced a case of periureteral varices with accompanying pyelitis, which was definitely confirmed by using the 3-dimensional rendering technique of the CT scan. Therefore, we report the case along with a brief review of the literatures regarding periureteral varices.