Objective:to review the clinical features, diagnosis and treatment of spinal Rosai-Dorfman disease(RDD).Methods:we conducted a systemic review and collected the cases reported from 2010. The key words were Rosai-Dorfman disease, spine/central nervous system. We screened both English and Chinese database. There were 43 reports finally included in the study, containing 52 cases. We distracted the information of interest and, subsequently, analyzed the harvested data using specific statistical software packages. The study focused on the summary and description of the clinical features, diagnosis and treatment and prognosis of spinal RDD.Results:The included articles reported 52 cases. The average age was 32.1±17.1 years (ranging from 6 to 76 years old). The ratio of male to female was 1.9/1. The median follow-up period was 19.9 months. The initial symptoms of 41 patients (78.8%) were spinal lesion-related. The cases with painless lymph nodes enlargement, other organ lesions and abnormal lab tests were 11.5%, 36.5% and 23.1%, respectively. The frequent infringed segments were cervical (43.1%) and thoracic (39.2%) spine. 53.2% lesions were dura-based, while 17.0% and 10.6% for bone and cord, respectively. Surgery was the mainstream of the treatment armamentarium, composing 83.7% cases, among which 46.3% underwent total resection. Cases only treated with radiotherapy, chemotherapy and steroids were 10.2%. Very Few cases remitted spontaneously (2.0%). The risk of recurrence and occurrence at other vertebral levels was 22.0%.Conclusion:It is rare for spinal involvement of RDD. This entity has no pathognomonic clinical and imaging features. RDD has a tendency of multi-organ involvement and recurrence. Surgery remains the mainstay of the treatment, but the efficacy of other adjuvant therapies is not sure. A wait and watch strategy is employed for asymptomatic patients.