Background/aims: Sclerosing cholangitis is a rare complication of Langerhans cell histiocytosis in children which can result in liver failure. This combination is even rarer in adults.
Case report: We report a 65-year-old female who developed sclerosing cholangitis 4 years after the diagnosis of Langerhans cell histiocytosis.
Conclusion: Sclerosing cholangitis caused by Langerhans cell histiocytosis is a rare condition in the adult population, but it has a high mortality. There is no definitive therapy other than liver transplantation. The long-term efficacy of liver transplantation remains unknown.
Keywords: Langerhans cell histiocytosis; Sclerosing cholangitis.