A case of transitional cell carcinoma of the ureter with inverted proliferation is presented. A 74-year-old man with the chief complaint of asymptomatic macrohematuria was referred for a suspicion of a ureteral tumor. Excretory urography demonstrated a filling defect with a round smooth contour in the right lower ureter. Urine cytology was negative for malignant cells. No bladder tumor was noted by cystoscopic examination. Under the clinical diagnosis of a right ureteral tumor, right total nephroureterectomy was performed. The gross specimen contained a 2.0 x 1.0 cm, polypoid, pedunculated and smooth-surfaced tumor. The pathological diagnosis was transitional cell carcinoma with inverted proliferation G2 much greater than G1. Malignant tumor with inverted proliferation in the ureter is very rare. In Japan, 8 cases of transitional cell carcinoma with inverted proliferation in the ureter, including our case, are reviewed.