We report on two cases of localized amyloidosis involving the ureter. The patients were a 64-year-old woman with right upper quadrant pain (case 1) and a 36-year-old woman suffering from left flank pain and intermittent gross hematuria (case 2). An intravenous pyelography of case 1 revealed multiple filling defects in the entire right ureter, whereas retrograde pyelography in case 2 showed diffuse narrowing in the mid and lower portions of the left ureter. Localized amyloidosis of the ureter was diagnosed in the two cases, and both had amyloid deposit in the renal pelvis and the urinary bladder in case 1, and in the contralateral ureter and the renal pelvis in case 2. Right nephroureterectomy was performed in case 1, but a segmental resection of the ureter with preservation of the kidney was administered in case 2. These two cases demonstrate that ureteral amyloidosis can be associated with amyloid deposition in the renal pelvis and the urinary bladder. Although ureteral amyloidosis is a rare occurrence, it should be considered in the differential diagnosis of ureteral obstruction to avoid unnecessary radical surgery.