Background: Extranodal follicular dendritic cell sarcoma (FDCS) is a rare neoplasm. That clinically mimics a wide variety of other abdominal tumors, and correct pre-operative diagnosis is often a challenging task. Case Report: A 49-year-old female presented with abdominal pain, abdominal fullness and underwent surgical excision of mesenteric tumor which was finally diagnosed as extranodal follicular dendritic cell sarcoma of mesentery on immunohistochemistry. Conclusion: When follicular dendritic cell sarcoma is suspected histologically, immunohistochemical stains for follicular dendritic cell differentiation are performed to avoid potential misdiagnosis.