Patients with end stage renal disease (ESRD) on maintenance hemodialysis invariably suffered from varying degrees of anemia. Erythrocytosis had been rarely reported in patients with chronic renal failure undergoing regular dialysis therapy. We described the case of a 43-year-old man with ESRD accompanied with erythrocytosis. Other causes of erythrocytosis were excluded by appropriate clinical studies such as polycythemia vera, renal cell cancer, hepatoma etc. Abdominal ultrasonography showed numerous renal cysts in patient consistent with acquired cystic disease of ESRD. Acquired cystic disease of the kidney in patient with ESRD can be associated with development of erythrocytosis. Peripheral serum erythropoietin level was maintained at normal range as measured by sensitive radioimmunoassay. We would like to indicate that erythrocytosis although rare in ESRD, should be associated with acquired cystic kidney disease in dialysis. Here, we report a case of erythrocytosis in long-term hemodialysis patient with the review of the literature.