Background: Pure red cell aplasia(PRCA) is a relatively rare disease, and a few of previous case reports in hemodialysis patients have described auto-erythropoietin antibodies-induced PRCA occurred due to recombinant human erythropoietin. However, a variety of factors contribute to the pathogenesis of PRCA and the underlying cause should be evaluated because cause-directed treatment is required. In this report, we introduce a case of carbamazepine-induced PRCA in an elderly male patient with ESRD on hemodialysis, whose anemia improved after discontinuation of the causative drug. Case presentation: A 70-year-old male patient with ESRD on hemodialysis for the past 8 years suddenly developed severe anemia, with his hemoglobin(Hb) level dropping to 6.2 g/dL. His Hb level had been maintained between 10.0 and 11.0 g/dL while receiving intravenously ESA therapy(epoetin-α, three times a week). Based on the bone marrow findings, pure red cell aplasia was diagnosed. Further examination to differentiate the cause of acquired PRCA did not reveal anything other than carbamazepine-induced PRCA. The results of ELISA tests for anti-EPO antibodies were also negative. We discontinued carbamazepine and switched to levetiracetam (Keppra®) for anticonvulsant effect. After three weeks later, his Hb level increased to 8.7 g/dL and he no longer required an RBC transfusion. However, after two months later, the Hb level decreased to 7.3 g/dL once more. We suspected erythropoietin deficiency due to chronic renal failure as the cause of anemia. While we gradually increased the ESA dose, his Hb level has remained stable at 11.0 g/dL. Conclusions: This is the first reported case of carbamazepine-induced PRCA in an elderly hemodialysis patient. The exact mechanism of drug-induced PRCA has not been elucidated; however, the disease can be cured without immunosuppressive therapy if the culprit drug is discontinued.