This article reviews the current status of ABO-incompatible kidney transplantation in the pediatric population. ABO blood type incompatibility between a donor and recipient was generally considered a contraindication to kidney transplantation because of the associated high risk for hyperacute rejection. However, due to a severe shortage of suitable cadaveric allografts, much effort has been made over the last decade to investigate whether successful and effective kidney transplantation is possible across the ABO blood group barrier. At present, ABO-incompatible kidney transplantation has been shown to be a valid alternative even for children with end stage renal disease. In this review, we will discuss protocols available for successfully performing ABO-incompatible kidney transplantation in children: (1) pre-transplant extracorporeal immunomodulation with removal of pre-existing anti-A and/or anti-B antibodies; (2) immunosuppressive therapy and anti-rejection therapy; (3) splenectomy and the associated infectious complication in asplenic children. Also, we will speculate regarding the mechanisms underlying accommodation following transplantation.