We report two cases of eosinophilic granuloma of the spine in which the first manifestation of the disease was an associated neural deficit. In addition, we review the literature for cases of histiocytosis X in which the initial diagnosis was based only upon the discovery of spinal lesions. Thirty-nine reported cases of what we designated as "symptomatic eosinophilic granuloma of the spine" were found. A review of all 41 cases revealed that most patients present with pain, muscular stiffness and/or neurologic symptoms, the disorder most often affects the cervical vertebrae, and a high incidence of osteolytic lesions (as opposed to vertebral collapse) are found. These findings vary from those of radiologic and clinical evaluations of classical, multifocal, histiocytosis X that involves the spine.