In a retrospective review of 242 cystectomy specimens performed for bladder carcinoma, ureteral carcinoma in situ was found in 14 patients (5.7%), unilateral in 12 and bilateral in 2. Pathology of the bladder specimen was pT4 (6 cases), pT3 (3 cases), pT2 (1 case), pT1 (3 cases), and pT0 (1 case). In the cystectomy specimen and in previous biopsies, they all had grade 3 tumor, and 85% had bladder CIS. Two patients were lost during follow-up. Seven patients (58.4%) died of metastatic disease without evidence of upper tract recurrence (UTR). Their average survival was 15.8 months (range 4-60). Five patients (41.6%) are alive after an average follow-up of 33.6 months (range 18-72 months). In one case an UTR appeared 53 months after cystectomy. In patients with ureteral CIS and long-term survival, a careful follow-up is advisable. The incidence of UTR is increased in this subgroup (8% global and 20% of survivors) but mortality is due to progressive bladder disease.