Amyloidosis of the ureter is a rare disease, distinguishing it from a neoplasm is difficult. A 64-year-old Japanese woman suffered from macrohematuria and left side hydronephrosis with ultrasound in 2014. Retrograde pyelography revealed no ureter tumor at that time. The patient had macrohematuria, left side hydronephrosis, and ureteral stenosis in the left ureter on retrograde pyelography. She was suspected of having a ureter tumor when she was 71 years old in 2021. The patient underwent ureteroscopy with biopsy. Pathological specimens contained the amyloid A component, based on immunohistochemistry staining. We diagnosed the patient with amyloid A amyloidosis of the ureter.