ABO incompatible allografting is contraindicated in most organ transplantations including heart because of the hyperacute and acute rejections caused by preexisting antibodies. However several reports showed that ABO incompatible organ transplantation could be managed successfully by plasmapheresis, antibody adsorption, immunosuppression, splenectomy, and so on. We experienced one success in ABO incompatible cardiac transplantation by means of plasmapheresis and immunosuppression. However, this does not justify heart transplantation across ABO blood group barriers. Because the effect of ABO incompatibility on continued acute rejection or chronic rejection has not been fully understood, long-term follow-up study is required.