A giant cell tumor occurs mainly in the proximal tibia, humerus, distal radius bone and the pelvic bone. It is rarely observed in such sites as the ribs and the temporal bone. The condition is primarily treated with surgical excision and functional reconstruction. The effect of chemotherapy on lung metastases and locally advanced giant cell tumors has remained unknown. We collected and analyzed the data of six patients with rare giant cell tumors located in the head and neck patients. After an average follow-up of 42.6 months after surgery (14 to 90 months), no local recurrence or metastasis was observed. We also collected and analyzed the data of five patients with metastatic giant cell tumors who were undergoing surgery for the primary tumor before; of three patients who had experienced multiple chemotherapy cycles, one had spontaneous regression, and one survived for long timer despite progression. The other two patients had their major metastatic lesions resected by surgery, and presented long-term survival during the follow up. In addition, this study reports one patient with locally advanced giant cell tumor of the rib, who has undergone successful surgical resection following two cycles of chemotherapy with ifosfamide and liposomal doxorubicin. Complete resection of the lesion at the head and neck is the key to relapse-free survival. The prognosis of lung metastases in patients with giant cell tumors is relatively satisfying. Neoadjuvant chemotherapy is also conducive to the surgery for locally advanced lesions and improvement of the quality of life.