Follicular dendritic cell (FDC) sarcomas of the nasopharynx are rare tumours; only seven cases have been reported in the English language medical literature. The authors present an eighth case, which occurred in a 70-year-old woman whose main complaint was nasal obstruction. It has been more than 10 years since FDC sarcoma was reported to occur in extranodal sites, and clinical and pathological characteristics of extranodal FDC sarcomas remain to be defined. The lack of a high index of suspicion is the main reason for misdiagnosis. The authors point out the difficulties in the diagnosis and management of this rare condition.