Objective To report a case of extramedullary plasmacytoma(EMP) and to explore the treatment options for this disease. Methods The clinic data and treatment plan of this patient were analyzed and the related literatures were reviewed. Results The clinical symptoms disappeared after radical surgery in this patient. It was definitively diagnosed as EMP with postoperative pathology, immunohistochemical, serum immunofixation electrophoresis. Oral CTd regime (compound cyclophosphamide 100mg QD, thalidomide 100mg QN D1-21, dexamethasone 15mg QW, one cycle every 28 days) was performed for 4 cycles since one month after the operation. There was no any evidence of recurrence or transformation of multiple myeloma(MM) during the 7-years follow-up. Conclusion For EMP at special site, surgical treatment should be selected as far as possible and the lesion should be resected as much as possible despite the difficulty and risk. Close follow-up should be conducted after the operation to prevent MM conversion. Low dose chemotherapy might be beneficial to the removal of possible residual lesions.