Follicular dendritic cell sarcoma arises from the follicular dendritic cells present in the lymph nodes, especially of the head and neck area. Rarely, extranodal sites may be affected including tonsil, the oral cavity, liver, spleen and the gastrointestinal tract. Here, we report a case of intra-abdominal follicular dendritic cell sarcoma presenting as a huge mass in the ileocecal region of a 24-year young woman.