Langerhans' cell histiocytosis (LCH) is a rare disorder of unknown etiology and pathogenesis. End-stage chronic liver disease is one presentation and orthotopic liver transplantation (OLT) has been reported in 17 cases, with variable resolution of LCH lesions postoperatively. We report a case of multisystem LCH with end-stage liver disease treated by OLT and review the overall results of OLT for children with LCH.