Background The problem of ABO-incompatible liver transplantation still remains unsolved in older children. In this article, we report on our experience of 6 successful ABO-incompatible liver transplantations in patients with biliary atresia. Material and Methods Six patients (ABO incompatibility type A→O:1 case, B→O:2 cases, A→B:3 cases) were enrolled in this study; 3 patients were aged approximately 1 year and the other 3 ranged in age from 9 to 24 years at the time of transplantation. Each patient received perioperative plasma exchange, until the anti–donor blood-type antibody titers became less than 1:16, and also systemic multidrug immunosuppressive therapy (cyclophosphamide, prednisolone, and tacrolimus). We applied the protocol of intraportal infusion therapy (local administration of prostaglandin E 1, steroid, and gabexate mesilate via a portal vein catheter), splenectomy, and rituximab administration for the older group. Results Both the patient and graft survival rates remain at 100%, with the follow-up period of the patients ranging from 12 and 123 months. Acute cellular rejection occurred in 2 cases, and both were steroid sensitive. There was no incidence of humoral rejection. Although all cases developed viral infection, all recovered uneventfully with the administration of antiviral agents. Conclusion ABO-incompatible liver transplantation can be performed with a low risk of humoral rejection or late biliary complications using this combined antirejection strategy, even in older children.