Follicular dendritic cell sarcoma(FDCS) is a rare malignant neoplasm arising from antigen-presenting cells in B-lymphofollicles and can be found at nodal or extranodal sites. We report a case of nodal FDCS of the neck. A 65-year-old female presented with a left neck tumor. Upon the initial diagnosis of metastatic cervical lymph node of unknown origin, neck dissection was performed. The postoperative pathological diagnosis was FDCS. Complete resection was performed, but we added radiotherapy as adjuvant therapy, because of reduction of locoregional recurrence rate. The patient is currently disease free after 18 months. With rare diseases such as FDCS, it is difficult to decide not only the diagnosis but also the appropriate treatment. In order to establish the treatment in future, more FDCS cases need to be accumulated. We hope this case report will help to establish the treatment.
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