Follicular dendritic cell sarcoma is a rare,malignant, non-lymphoid cell-derived tumor that originates from B-lymphoid follicles of nodal and extranodal sites. It has been reported in around 100 cases in the literature,mostly for the head and neck region. Surgery and radiotherapy were considered the mainstay of treatment for localized disease. As for the metastatic setting,classic lymphoma and sarcoma regimens were previously tested with dismal responses. Patient and Methods: In this report, we examined imatinib in combination with gemcitabine and cisplatin for treating a male patient with metastatic follicular dendritic sarcoma to the liver and lung. Results: The patient achieved complete pathological remission confirmed by positron emission tomography(PET) scan after 8 cycles. Conclusion: Our case is the first to report a role for imatinib and to show complete pathological remission in this rare disease. An imatinibbased combination could serve as a good therapeutic option for such cases.