To examine the association of response to rituximab and the incidence of antibody-mediated rejection in preconditioning of rituximab and plasma exchange without post-transplant plasmapheresis in patients undergoing ABO-incompatible living kidney transplantation. A total of 115 patients who underwent ABO-incompatible living kidney transplantation at Tokyo Women's Medical University Hospital, Tokyo, Japan, were divided into two groups based on the response to rituximab: good response (n = 75) or poor response (n = 40). The rituximab good response and poor response patients were defined as patients whose CD19+ cells were non-detected (0%) and detected on the day of transplantation (2-5 days, median 3 days, after rituximab administration), respectively. Rituximab response and anti-A/B blood antibody titer after plasmapheresis were significant risk factors for antibody-mediated rejection (P = 0.036, 0.045, respectively). The occurrence of antibody-mediated rejection was higher in the poor response group than in the good response group (22.5% vs 8.0%; P = 0.028). The 14-day, 3-month and 1-year cumulative incidence of antibody-mediated rejection was 2.7%, 5.3% and 8.0% in the good response group, and 17.5%, 20.0% and 22.5% in the poor response group after ABO-incompatible living kidney transplantation. The patient survival was not significantly different between the two groups. However, graft survival 1 month after transplantation was lower in the poor response group. There is no significant difference in graft function and in the incidence of complications, including infection, after transplantation between the two groups. Antibody-mediated rejection after ABO-incompatible living kidney transplantation was significantly associated with the response to rituximab in our preconditioning protocol.