The ABO-incompatible living-donor kidney transplantation was developed in Japan in 1989. Currently, most transplant physicians and surgeons have noted that outcomes are unexpectedly excellent, and no hyperacute rejections have been reported since 2001. In the registry of the Japanese ABO-Incompatible Kidney Transplantation Committee, the data of 2434 ABO-incompatible living-donor kidney transplants were collected from 120 Japanese kidney transplant centers. Overall patient and graft survival rates were 97% and 94% at 1 year, 93% and 86% at 5 years, 90% and 71% at 10 years, and 73% and 52% at 20 years. The patient survival and graft rates in 2001 to 2012 were 93% and 81%, which were significantly better than 83% and 55% reported in 1989 to 2000. The addition of novel immunosuppressive treatments has improved results. Azathioprine has been replaced by mycophenolate mofetil since 2000 to 2001, and basiliximab and rituximab were introduced in 2002 and 2004. The titer of antidonor blood group antibody before transplantation was not correlated with graft survival in 2001 to 2012. De novo antibodies against vascular endothelium of peritubular and glomerular capillaries seemed to be more important than natural antibodies against red blood cells. Therefore, recipients with antidonor blood group antibody titers < 1:128 did not require antibody-removal procedures such as plasmapheresis or immunoadsorption. In particular, children (regard less of their peritoneal dialysis status) do not need to be catheterized for plasmapheresis or immunoadsorption. It is better to avoid the risks of catheterization and antibody removal procedures in children with end-stage renal failure.