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.