Follicular dendritic cell sarcoma is a rare neoplasm characterized by the proliferation of tumor cells demonstrating immunohistochemical features of normal follicular dendritic cells. There is no sex predilection, and patients are diagnosed over a wide age range, with a median age of 50 years. It is more common in the lymph nodes, but extranodal cases are also reported, affecting the gastrointestinal tract, soft tissue and the lungs most often. In the oral and maxillofacial region, the tonsils and the soft palate are the most commonly affected subsites. The nodal presentation of the tumor is usually characterized by an asymptomatic swelling with an indolent course, while in the tonsils and soft palate, the neoplasm is more frequently described as a unilateral ulcerated mass with or without associated pain. Microscopically, the neoplasm is composed of spindle to oval-shaped cells distributed in variable patterns, including a diffuse growth pattern or vague nodules. Follicular dendritic cell sarcoma has been treated by complete surgical resection with an excellent overall survival rate. Local recurrences occur in approximately 40% of the cases, and distant metastases may take place in up to 25% of the patients, usually involving the lungs, liver, adrenal glands, and ribs.