Introduction: We summarized our experience with ABO-incompatible living kidney transplant recipients from spousal donors receiving rituximab. Patients and Methods: Between June 2006 and December 2014, 82 patients with end-stage renal disease underwent living donor kidney transplantation at Osaka City University Hospital, of which 23 cases were ABO-incompatible transplantation between spouses with rituximab induction. We analyzed these recipients, focusing on their immunosuppressive protocols, frequency of acute rejections, and patient/graft survivals. Results: Patient and graft survival rates were 100%. The incidence of acute cellular rejection (ACR) was 30.4%. One patient experienced antibody-mediated rejection (AMR) and intractable ACR, 2 had AMR, and 2 had intractable ACR episodes that were treated using thymoglobulin. Conclusions: This study demonstrated that ABO-incompatible kidney transplantation between spouses using rituximab is a radical but effective treatment for end-stage renal disease. However, this procedure could be immunologically high risk due to ABO-incompatibility and poor histocompatibility.