To explore the clinical manifestations, pathological features, immunohistochemical features, imaging findings, differential diagnosis treatment and prognosis of follicular dendritic cell sarcoma (FDCS), and aims to deepen the knowledge of follicular dendritic cell sarcoma among clinicians. The clinical and follow-up data of a patient with the right neck FDCS from our hospital were reported, and we have clear the diagnosis and treatment with domestic and foreign literature. The clinical manifestation was a painless mass with slow growth in the right neck and local recurrence after surgery. Microscopic examination showed the structure of the lymph nodes was damaged; tumor cells admixed with abundant lymphocytes in the background arranging in fascicles, whorled, storiform or diffusions. Tumor cells were spindle or oval in shape, with abundant cytoplasm, often visible nucleoli. Immunohistochemical staining showed the patient that S-100 (+), CD21 (+), CD23 (+). Follicular dendritic cell sarcoma is a rare malignant tumor and could be easily misdiagnosed. Due to its low incidence, easily recurrence and metastasis, have something difficulty in imaging diagnosis, using reasonable application of immunohistochemical markers combined with pathological tissue chemistry are the necessary condition to avoid misdiagnosis.