The optimal management in living donor liver transplantation using an ABO incompatible donor with a high isoagglutinin titer is still uncertain. Our patient was a 20-year-old woman with fulminant hepatitis. The only available donor was her 54-year-old father-in-law of an incompatible blood type. The initial isoagglutinin titer was 2048x. She received 375 mg/m2 of anti-CD20 antibody 3 days before the living donor liver transplantation with concomitant splenectomy. Despite daily plasma exchanges after transplantation, the isoagglutinin titer started to shoot up to its maximum value of 2048x, with a sudden decline in the bile output. High-dose intravenous immunoglobulin (0.6 g/kg) was given after the plasma exchanges; thereafter, her liver function tests stabilized without a further increase in the isoagglutinin titer. We showed the effectiveness of high-dose intravenous immunoglobulin for the management of the rebound elevation of isoagglutinin titer. The combination of anti-CD20 antibody and daily plasma exchanges seemed ineffective for such a situation. This strategy might be another management option for ABO incompatible liver transplantation.