Objective To perspectively evaluate the prevalence and clinical characteristics of acquired cystic kidney disease and renal cell carcinoma in hemodialysis patients. Methods The prevalence and clinical characteristics of 38 patients with acquired cystic kidney disease (ACKD) and renal cell carcinoma in a total of 136 consecutive hemodialysis patients reviewed and analyze. Result Thirty-eight (27.94%) of 136 maintenance hemodialysis patients had ACKD, two patients (5.26%) had histologically proven localized renal cell carcinoma (unilateral). Conclusion The prevalence of ACKD is positively related to the duration of dialysis, the high prevalence of renal cell carcinoma is related to ACKD in maintenance hemodialysis patients.