We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65 %), visual disturbance in four (16 %), polyurea-polydipsia in three (13 %), and progressive weakness in all extremities in one patient (4 %). Neurological examination revealed no abnormality in sixteen patients (70 %), cranial nerve palsy, visual field defect and optic atrophy in six (26 %) and paraparesis in one (4 %). Tumoral mass was found to be located on the cranial vault (65 %), in the suprasellar region (21 %) and in the spinal column (8 %). The cranium and spinal column were both involved in one patient. All patients underwent surgery. craniectomy with grossly total tumor excision plus cranioplasty (65 %), craniotomy with subtotal tumor excision (26 %), and vertebrectomy with grafting (13 %) were performed. The clinical, radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.