Chordoma is a low-grade malignant tumor originating from the remnants of the embryonic notochord that grows slowly and aggressively, has a long course, and generally rarely metastasizes. It is often found on the slope of the skull base, in the saddle area and in the sacrococcygeal region of the spine. Chordoma of the skull base is deep and invades important structures such as surrounding bone, cranial nerves and large blood vessels, making total resection difficult. In recent years, with the development of neuroimaging technology, microsurgery technology, and stereotactic radiotherapy technology, significant progress has been made in the diagnosis and treatment of skull base chordoma, especially in radiosurgery. The clinical characteristics of skull base chordoma and the current status of research in radiosurgery are reviewed in this article.