Objective To explore the therapeutic benefit of liver transplantation （LT）on progressive familial intrahepatic cholestasis （PFIC）. Methods The clinical data of 5 cases with PFIC who underwent liver transplantation at Huashan Hospital affiliated to Fudan University from October 2015 to April 2018，were analyzed retrospectively. There are PFIC Ⅱ patients in 3 cases and 2 PFIC Ⅲ patients in 2 cases ；there were 3 cases male and 2 cases female with a median age of 34 months（9 ～ 102 months）；living donor liver transplantation with left lateral segment patient in 4 cases and classical orthotopic liver transplantation in 1 case. The clinical data， including pre-operative basic features， surgical situation， postoperative management and outcomes were analyzed to evaluate the curative effect of LT. Results All the surgeries achieved success and the liver function recovered smoothly. After the operation，1 case was complicated with lymphatic leakage and 1 case with intrahepatic bile duct dilation. One case was infected by the hepatitis B virus， cytomegalovirus and EB virus. All the children and grafts were alive after median follow up duration of 23 months（4 ～ 34 months）. The clinical manifestations of jaundice and pruritus were gradually vanished. No patient suffered from apparent acute or chronic rejection and vascular complications Conclusions LT is the definitive treatment for PFIC as it could correct the genetic defect.