Background: Patients with maintenance hemodialysis (HD) have been recognized as a high risk group for cancer. The aim of this study was to verify the incidence and oncological outcomes of patients on maintenance hemodialysis with renal cell carcinoma (RCC) compared with RCC patients without renal dysfunction. Methods: We have been carried out annual screening for renal mass for the hemodialysis patients.The oncological outcome of pT1N0M0 RCC detected by annual screening of CT imaging were retrospectively reviewed in 1217 patients with HD between January 2002 and December 2010 at Oyokyo kidney Research Institute and Hirosaki University Hospital, Hirosaki, Japan. Overall and cancer specific survival was compared with age matched 106 of pT1N0M0 RCC patients without renal dysfunction who performed radical nephrectomy at the same periods. Results: Among the hemodialysis patients, 14 RCCs were incidentally detected by screening CT examinations and RCC detection rate in HD patients was 0.27% per year. Total 119 pT1N0M0 RCC patients (13 with HD group, 106 normal kidney function group) were incidentally detected by regular abdominal CT imaging without symptoms. There were no significant differences on age and gender between HD and normal kidney function group. Cancer specific survival after tumor diagnosis was not different, but overall survivals were significantly superior in patients without renal dysfunction compared to those with chronic renal failure (P < 0.0001). Cancer specific and Overall 5-year survival was 95% and 96% in patients without renal dysfunction, 92% and 54% in those with chronic renal failure. Conclusions: There was no significant difference in cancer specific survival of pT1 RCC between the two groups. However, overall survival was significantly worse in HD patients. Oncological effectiveness of annual CT screening for the HD patients was not evident in the present retrospective study.