Follicular dendritic cell sarcoma is one of the rare and aggressive neoplasms originating from follicular dendritic cells of lymphoid tissues. It commonly presents as asymptomatic lymphadenopathy, but extranodal involvement such as oral cavity, mediastinum, liver, and spleen are also reported. The disease often has an indolent course. Current knowledge on its pathogenesis is limited and due to its rarity, follicular dendritic cell sarcoma has no definite treatment strategy at present. Here we report a case of a 24-year old male with follicular dendritic cell sarcoma of neck, treated with wide local excision, post operative radiation and chemotherapy, and developing pulmonary metastasis after a disease-free period of 15 months.