We report a case of ureteral cancer, an uncommon disease entity, which presented as a radiolucent ureteral stone, a common problem for urologists. This 72-year-old female suffered from acute left renal pain with gross hematuria. Intravenous pyelography suggested a radiolucent stone in the left-side distal ureter with obstructive uropathy. After failure of shock-wave lithotripsy, abdominal computed tomographic (CT) scanning showed that the left-side ureteral obstruction persisted with no ureteral stone. Diagnostic ureteroscopy identified a papillary tumor in the left distal ureter, but the biopsy was negative for malignancy. The patient underwent a left total nephroureterectomy with removal of the bladder cuff. The pathology was compatible with a locally advanced ureteral transitional cell carcinoma. She had a smooth postoperative recovery with adjuvant chemotherapy. We concluded that the possibility of an underlying ureteral tumor must be kept in mind when dealing with a possible radiolucent stone.