Follicular dendritic cell sarcoma (FDCS) of the nasopharynx is a rare malignant tumor that has been described in only a few case reports, and its differential diagnoses include diverse clinicopathologic entities. FDCS is often initially misdiagnosed, especially when examining small biopsy specimens. We herein report a case of FDCS arising in the nasopharynx that was initially misdiagnosed as a nerve sheath tumor. A 44-year-old woman presented with persistent obstruction of the left nasal cavity and underwent an excisional biopsy. The specimen demonstrated morphologic and immunohistochemical features of FDCS. In situ hybridization for Epstein–Barr virus-encoded RNA was negative. The patient was treated with chemotherapy and radiotherapy. The sarcoma recurred near the original site more than 3 years after the initial treatment and was completely resected. At the time of this writing, the patient had remained disease-free for 1 year after resection. This case is being reported to improve the clinical recognition of FDCS.