ABO-incompatible intestinal transplantation has rarely been performed due to poor patient outcomes. Herein we present a case of successful ABO-incompatible intestinal transplantation with a 2-year follow-up. A 16-year-old female with a history of extensive bowel resection received an ABO-incompatible living donor bowel graft from her father (blood type AB graft into a type A recipient). Posttransplant immunosuppression consisted of an initial anti-CD20, plasmapheresis/intravenous immunoglobulin before transplantation, followed by an anti-thymocyte globulin (ATG) induction and splenectomy, and maintenance with tacrolimus and prednisone. Her postoperative course was remarkable for a single episode of rejection on day 14 which responded promptly to treatment with methyprednisolone and ATG. Three months after transplantation, the patient developed an abdominal abscess requiring open surgical drainage. No viral infections were encountered. Posttransplant anti-B antibody titers and anti-B7 donor-specific antibody levels remained low. At a 2-year follow-up, the patient showed a progressive weight gain of 5.0 kg. This case illustrates that ABO-incompatible living-related bowel transplantation is immunologically feasible and is associated with good outcomes for the recipient. The management of blood type antibodies and the use of adequate immunosuppression in the early period of the procedure may be the keys to the success of future cases.