Background: Langerhans cell histiocytosis, formerly known as histiocytosis X, is characterised by clonal proliferation of pathologic cells resembling Langerhans cells. Langerhans cell histiocytosis is commonly localised in the bones of the skull or in the skin; however, a great variety of foci has been described.
Case: A general practitioner referred a 25-year-old man to the neurologist because of progressive pain in the neck that had arisen spontaneously.