Irie R, Nakazawa A, Sakamoto S, et al. - This study was sought to elucidate the prognosis of children with congenital hepatic fibrosis (CHF) who received living donor liver transplantation (LDLT) from donors who might be heterozygous carriers of a hepatorenal fibrocystic disease. Researchers included a total of 14 children with CHF who underwent LDLT at our center. The histological and radiological findings of the donor livers and complications in the recipients following the liver transplantation were analyzed. It was demonstrated that the prognosis of recipients who received a LDLT from their parents for CHF was excellent. Nevertheless, they found abnorma morphology of half the donor livers. In addition, careful follow-up is required to warrant long-term graft survival.