Abstract
The histiocytic disorders are pathological diagnosis and rarely affects spine. The spinal involvement is characterized by lytic lesions and painful symptoms. Isolated intradural extramedullary involvement is rare presentation. A 15-year-old female patient presented with nontraumatic cervical compressive myelopathy and was operated electively with preoperative diagnosis of meningioma. The histopathology was surprisingly Langerhans cell histiocytosis. In lineage of histiocytic development, the Langerhans cells develop into matured dendritic cells and lose its Birbeck granules and CD1a antigenicity. With the understanding of histiocyte lineage system, the disorders concerned with central nervous system are classified into dendritic cell disorders and macrophages-related disorders. In this article, we have discussed on histiocytic disorders of central nervous system and management guidelines in case one comes across such histopathology.