We investigated the clinicopathological features of 62 patients with transitional cell carcinoma of the renal pelvis and ureter. Four patients had been treated for bladder cancer. Among 58 patients without precedent bladder cancer, 6 had coexistent bladder cancer and bladder cancer subsequently developed in 13. The 5-year cause-specific survival rate was 33% in cases with coexistent bladder cancer, 75% in those with subsequent bladder cancer and 62% in patients without association of bladder cancer. Distant metastases were found in 23 of 62 (37%) cases, the most frequent site being lymph nodes. The site of the primary tumor (renal pelvis and/or ureter) and the pathological findings such as grade, stage, type of infiltration, venous and lymphatic invasion, were significantly correlated to cause-specific survival. Multivariate analysis showed the most influential factors to be the type of infiltration and the site of the primary tumor. Therefore, patients with INF beta or gamma tumors both in the renal pelvis and ureter had a poor prognosis. However, association of bladder cancer was not related to survival.