Primary maxillofacial chordoma is extremely rare. We herein report a very rare case of a recurrent maxillofacial chordate tumor that was diagnosed in a 56-year-old woman who underwent three tumor resections. After surgical treatment, the patient healed well with an Eastern Cooperative Oncology Group score of 1. She was discharged to a local hospital for adjuvant radiotherapy. Close follow-up was ongoing at the time of this writing. Radical surgery and adjuvant radiotherapy remain the main treatment strategies for chordoma. Postoperative radiotherapy is particularly important. Our experience is to administer a total dose of 50 Gy to a clearly delineated target. If appropriate comprehensive treatment is available, distant metastasis of primary chordoma is rare, and neck dissection is therefore not generally recommended. Neck lymph node dissection is generally not recommended.
Keywords: Chordoma; case report; maxillofacial; radiotherapy; recurrence; resection.