Skull base chordoma is a locally invasive tumor, which can expand in different directions with the invasion of skull base. Although they are histologically low-grade, they easily invade peripheral blood vessels and nerves, which makes it almost impossible to completely remove them in most cases, which leads to residual tumors after surgery. At present, the main treatments are surgery and radiotherapy, but the recurrence rate is high and the prognosis is poor. This article reviews the latest research progress of skull base chordoma, so as to provide help for the treatment of skull base chordoma.