Owing to the severe shortage of cadaveric grafts in Japan, we have performed ABO-incompatible living donor kidney transplantation since 1989. This study assessed short- and long-term outcomes in 441 patients who received ABO-incompatible living donor kidney transplants between January 1989 and December 2001. We compared our results with historical data from 1055 recipients of living kidney transplantation. Overall patient survival rates 1, 3, 5, 7, and 9 years after ABO-incompatible transplantation were 93%, 89%, 87%, 85%, and 84%, respectively. Corresponding overall graft survival rates were 84%, 80%, 71%, 65%, and 59%. After ABO-incompatible transplantation, graft survival rates were significantly higher in patients 29 years or younger than in those 30 years or older and in patients who received anticoagulation therapy than in those who did not receive such therapy. There were no significant differences between A-incompatible and B-incompatible recipients with respect to clinical outcomes. The graft survival rate at 1 year in the historical controls was slightly but not significantly higher than that in our recipients of ABO-incompatible transplants. We conclude that long-term outcome in recipients of ABO-incompatible living kidneys is excellent. Transplantation of ABO-incompatible kidneys from living donors is a radical, but effective treatment for end-stage renal disease.