Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We
report on a rare case of its primary oral manifestation that was treated
successfully with the BRAF-specific agent, vemurafenib, after insufficient
standard LCH treatment. This case underlines the importance of proper diagnosis
and the evaluation of targeted therapy as a valuable tool in LCH treatment.
Furthermore, the close collaboration of surgeons, oncologists, and dentists is
mandatory to ensure adequate treatment, restore the stomatognathic system in
debilitating post-treatment situations, improve quality of life, and ensure
effective disease control in infants and young patients.