Abstract: Chordoma, as a highly malignant primary tumor, originates from residual embryonic spinal cord tissue, with a high recurrence rate and poor prognosis. Although current research has found that multiple molecules and signaling pathways, including the Brachyury gene, are involved in the occurrence and development of spinal cord tumors, their etiology is still unclear. Due to the strong metastasis and invasion of surrounding tissues, the treatment of spinal cord tumors remains a major challenge that urgently needs to be addressed. Identifying the key molecules and signaling pathways involved in the occurrence and development of spinal cord tumors can not only help to gain a deeper understanding of the pathogenesis of spinal cord tumors, but also provide new directions for improving the current status of spinal cord tumor treatment. This article reviews the research progress of chordoma from the aspects of molecular signaling, clinical diagnosis and treatment.