Follicular dendritic cell sarcoma is a rare mesenchymal neoplasm arising from follicular dendritic cells and accounts for only 0.4% of soft tissue sarcomas. Most of the follicular dendritic cell sarcomas arise from lymph nodes. However, at least one third cases occur at extranodal sites. Clinico-pathological misdiagnosis is common in this disease because of its similarity in clinical presentation, histopathological findings and immunohistochemical markers in initial evaluation to many malignancies. The genetic alterations leading to tumourigenesis in follicular dendritic cell sarcoma are unknown. The disease is considered under intermediate grade malignancy, but it has significant locoregional recurrence and distant metastatic potential. The clinical history, different pattern of treatments, and response to treatments is unclear due to insignificant data. But to advance in diagnostic assays and few chemotherapeutic agents helps in accurate diagnosis and better treatment of follicular dendritic cell sarcoma.