Abstract. We describe a case of follicular dendritic cell sarcoma (FDCS) of the tonsil in a 59-year-old woman. She was successfully treated with excision of the mass and postoperative radiation therapy. According to our review of the literature, only 25 cases of extranodal FDCS in the head and neck have been previously reported, including only 10 cases that involved a tonsil. We briefly review these earlier reports, and we discuss the diagnosis and management of FDCS.