Follicular dendritic cells are derived from the stroma. They are present in the germinal centers of lymph nodes and involved in antigen presentation, proliferation and differentiation of B cells. Follicular dendritic cell sarcoma (FDCS) is a relatively rare malignant tumor and its biological behavior is usually indolent. Most patients may be cured by complete surgical resection. Local recurrence may be present in >40% of cases and ~25% of cases may have metastasis. Recurrence or metastasis may not occur until numerous years later. In previous case reports, patients with splenic FDCS recovered or were stable after surgical resection of the tumor. In the present case, the tumor was initially located at the lower end of the spleen and radical resection was performed by laparoscopy without lymph node and distant organ metastasis. However, multiple metastases occurred after surgical resection. The patient received chemotherapy and radiotherapy, but his condition did not improve. The time from discovery of spleen tumor to death of the patient was 9 months. The purpose of the present case report was to raise clinicians' awareness of the disease, provide information about the treatment process and incite research on splenic FDCS and its prognosis.