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We explored the presence of <jats:italic>BRAF<jats:sup>V600E<\/jats:sup><\/jats:italic> mutation in our cohort of patients with LCH and cholestasis, sclerosing cholangitis, or liver fibrosis that presented resistance to chemotherapy. The <jats:italic>BRAF<jats:sup>V600E<\/jats:sup><\/jats:italic> mutation was detected either in the diagnosis (skin and bone) or liver biopsy in our cohort of 13 patients. 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