Abstract: Intramedullary spinal cord metastasis (ISCM) is a dangerous metastatic disease among advanced cancer patients with tumor metastases in the spinal cord because it compresses the spinal cord or nerve root. This phenomenon can cause pain, hypoesthesia, or numbness, especially in severe cases, leading to paralysis of the body and significantly reduced quality of life. With the increase in tumor morbidity and the application of new imaging diagnostic techniques in clinical practice, the detection rate of ISCM has significantly improved. Unfortunately, a consensus on ISCM treatment remains lacking to date. In addition, the role of radiotherapy in previous ISCM treatment strategies is limited. However, the development and improvement of radiotherapy have rendered this technology the main treatment for patients who cannot be treated surgically or are insensitive to chemotherapy. This article reviews the progress in imaging diagnosis and treatment of ISCM.