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Due to an extreme shortage of cadaveric kidneys, many centers in Japan successfully performed ABO-incompatible kidney transplantations using plasmapheresis, splenectomy and immunosuppression. Recently, a protocol including anti-CD20 monoclonal antibody (rituximab) and antigen-selective immunoadsorption has been used for ABO-incompatible transplantation in Europe. In Korea, ABO-incompatible kidney transplantation has been rarely performed. We report an experience of successful ABO-incompatible kidney transplantation using plasmapheresis and rituximab. The patient was a 32-yr-old female suffering from chronic renal failure, and her blood type was O, Rh+. The donor was her husband, and his blood type was B, Rh+. A combination therapy including 5 times of plasmapheresis starting from 10 days before transplantation with 2-day interval, intravenous gammaglobulin, rituximab at 2 weeks before transplantation and potent immunosuppression successfully decreased the titers of anti-A and anti-B antibodies to 1:2 and 1:1, respectively. The kidney transplantation was successful without any sign of hyperacute or acute rejection.