Abstract: Objective To improve our understanding of follicular dendritic cell sarcoma（FDCA） and study its treatment plan. Methods One case of metastatic FDCA of lung admitted to our department was reported with its related literature reviewed.Its diagnosis and treatment methods were analyzed. Results Early metastatic FDCA of lung occurred and progressed rapidly in the case who received radiotherapy and chemotherapy with cyclophophane,vincristin,and predinisone,etc,after 2 local operations.The tumor was removed with lymph nodes around it cleared,which was finally diagnosed as a metastatic FDCA of lung by pathology. Conclusion FDCA is a rare tumor with a high malignancy and should be systematically treated.