Infants with ALF occasionally have the most urgent need for a liver transplant. In urgent situations for liver transplantation, LDLT has been advocated. Between July 1995 and April 2004, medical records of 15 infants undergoing LDLT for ALF of unknown etiology were reviewed and their outcomes were compared with infants undergoing LDLT for other liver diseases. They received LLS (n = 9), MS (n = 3), and RMS (n = 3) grafts. Eight technical complications occurred, with a similar incidence for LDLT and the other liver diseases. On the other hand, the liver biopsies after LDLT showed a significantly higher incidence of ACR with centrilobular injuries. Ten patients died after primary LDLT because of refractory rejection (n = 6), rotavirus infection (n = 2), chronic rejection (n = 1), and surgical complications (n = 1). With a median follow-up of 7.0 months, five-yr graft and patient survival rates were 17.8% and 26.7%, respectively. In conclusion, the outcome of LDLT for ALF in infants, especially cases with unknown etiology, was unsatisfactory and refractory rejection often led to liver failure. From our observation the centrilobular changes were characteristics of ACR in infantile LDLT for cryptogenic ALF.