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At this time, everolimus was administered, and soon after that, liver enzyme levels had gradually improved. Currently, the patient is doing well for 44 months to date without any abnormal findings. The maintenance target trough concentrations were tacrolimus 5 ng/ml and everolimus 3 ng/ml. Our case demonstrated the effect of rescue therapy using everolimus for ACR following pediatric LDLT. Further studies are needed to assess the role of everolimus in pediatric liver transplant recipients suffering from ACR.Biliary papillomatosis is a rare disease of the bi