Follicular dendritic cell tumour/sarcoma is a rare tumour involving lymph-node or extra-lymph-node sites; review of the literature reveals very few cases of follicular dendritic cell tumour, probably since, in the past, the disease has often been mistaken for other neoplasms: low differentiated carcinomas and fusate cell carcinomas, sarcomas, melanomas, thymic neoplasms, Castle carcinoma and other dendritic cell tumours (especially interdigital cell tumour/sarcoma). In the case described here, attention is focused on the diagnostic difficulties and on the therapeutic profile, comparing data with those reported in the international literature.