目的：手术治疗颅颈脊索瘤。方法：根据肿瘤的部位不同而分别采用：经腭、颈侧、侧颅底三种手术径路。结果：４例中有３例术后半年至１年死亡，１例术后１５年仍健。结论：颅颈脊索瘤是一种少见，生长缓慢，病程长的肿瘤，因压迫吸收骨质，可凸入口咽、鼻咽、颅内、眼眶等处，引起相应症状。ＣＴ和ＭＲＩ可显示肿瘤部位、范围。诊断有赖于病理。虽属良性，但因其具有胞核深染，核分裂，畸形核，多形细胞及向软组织浸润，少许可有血行实质性脏器的转移。免疫组化有类似软骨肉瘤的特征，因此现视为低度恶性的肿瘤。其对放射治疗不敏感，手术为主要治疗手段。然而往往因肿瘤侵蚀椎旁间隙，椎间隙，椎管，硬脑膜而常不能切除彻底，导致复发，因此早期诊断与治疗有重要的意义。 Objective: Surgical treatment of cranial and cervical chordoma. Methods: According to the different parts of the tumor were adopted: the palate, neck, skull base three surgical approach. Results: Of the 4 cases, 3 died after 6 months to 1 year and 1 case remained healthy after 15 years. CONCLUSION: Cochlear chordoma is a rare, slow-growing, long-course tumor that can cause symptoms by compressing and absorbing bone and protruding into the oropharyngeal, nasopharynx, intracranial, orbit. CT and MRI can show the tumor site, the scope. Diagnosis depends on the pathology. Although benign, but because of its deep-stained nuclei, mitosis, deformity nuclear, pleomorphic cells and soft tissue infiltration, a little blood may have a substantial organ metastasis. Immunohistochemistry has similar features of chondrosarcoma, it is now considered a low-grade malignancy. It is not sensitive to radiation therapy, surgery as the main treatment. However, often due to tumor erosion paravertebral space, intervertebral space, spinal canal, dura and often can not be completely removed, leading to recurrence, so early diagnosis and treatment of great significance.