A 78-year-old man visited our hospital with a chief complaint ofmacrohematuria in January 2006. Ultrasonography and computed tomography showed left hydronephroureter due to the enhanced tumor which was 10 mm in diameter in the lower ureter. Urine cytology was Class II. Retrograde pyelography could not be performed because of severe pain and hematuria. We considered the possibility of the malignant neoplasm and performed left total ureteronephrectomy in March 2007. The mucosa of the ureter was thickened and sclerosed. Microscopic appearance of the surgical specimen shows non-structural fibrosis and stained red with Congo-red stain. So amyloidosis of the ureter was suspected. After the systemic examination, the diagnosis was localized amyloidosis of the ureter. After 8 months, recurrence has not been pointed out.