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Follicular dendritic cell sarcoma (FDCS) is a rare neoplasia composed of spindle or oval cells with follicular dendritic cell differentiation, usually occurring in lymphoid tissue. In this report, we present a case of FDCS of the small intestine with liver metastasis.A 19-year-old female presented with recent onset of left upper abdominal pain. Abdominal computed tomography scan showed a large tumor mass in the liver lateral segment with compression to the pancreas upper part, and a smaller mass in the terminal ileum, respectively. High serum levels of amylase and lipase were noted. Resection of the tumors was performed. Microscopically, both tumors consisted of ovoid to spindle-shaped nuclei cells admixed with some lymphocytes arranged in fascicles, whorls, storiform arrays. Immunohistochemistry demonstrated that the tumor cells were positive for follicular dendritic cell markers, including CD21, CD23, and CD35. Epstein–Barr virus encoding small RNA (EBER; Inform EBER probe; Ventana Medical Systems, Tucson, AZ) in situ hybridization was negative.According to the clinicopathological features, diagnosis of FDCS of intestinal origin was made.Resection of tumors located in the liver and at the small intestine was performed. After the operation, patient received adjuvant vinblastin chemotherapy.There was no evidence of recurrence at 8-month follow-up.It was unusual for FDCS of intestinal origin with liver metastasis and expressing with high serum levels of pancreatic enzymes.