Primary localized amyloidosis of the ureter and bladder is a very rare condition. CT and cystoscopic findings are indistinguishable from malignant neoplasm or inflammatory lesions. We report a case of unilateral distal ureteral thickening caused by primary localized amyloidosis. MR image features of hypointensity on T2-weighted images and mural enhancing pattern on contrast enhanced T1-weighted images can be helpful for diagnosis of ureteral amyloidosis.