Chordoma is a low-grade malignant tumor originating from residual notochordal tissue, which is relatively rare and is more common in males than females between the ages of 20-40. The tumor is located outside the dura mater and can grow expansively in all directions, both intracranially and extracranially. Surgery is the first treatment option, but the tumor is widely encroaching on the skull base and often adheres tightly to the nearby important structures, so the total resection rate is low, but we should strive for major or subtotal resection to relieve symptoms and prolong survival. According to the main location of the tumor, different surgical approaches can be chosen, such as transfrontal epidural approach, infratemporal approach, transtentorial sinus approach, transoral or posterior lateral (transcondylar) approach. In principle, the tumor compressing the brainstem should be resected first, and then further resection of the tumor can be considered to reduce the load of radiation therapy. Complete resection combined with high-dose radiation therapy can achieve good therapeutic results, and recurrence can be delayed when conventional radiation therapy is combined with palliative or decompressive surgical treatment. One of the stereotactic radiotherapy treatments, heavy particle radiotherapy, can effectively reduce the damage to surrounding tissues and is suitable for slower growing chordoma. Chordoma is insensitive to conventional chemotherapy, and molecularly targeted drug therapy has a certain inhibitory effect on chordoma and can promote apoptosis of tumor cells. Chordomas are pathologically mostly benign lesions, but have significant malignant behavior and a high recurrence rate after surgery.