TCS (spinal cord cord embolism syndrome) is a series of neurological dysfunctions and concomitant malformations due to various congenital or acquired causes of spinal cord involvement. The spinal cord can be impinged at any site, including the cervical, thoracic, and lumbosacral medulla, but the most common site of impingement occurs in the lumbosacral medulla, resulting in an abnormally low cone position, which is the most common presentation of TCS. TCS usually occurs in children, especially infants, and is slightly more common in females than males, with an incidence of up to 1 in 4000. In TCS, neurological impairment can occur due to prolonged stretching of the spinal cord, especially the conus, which can lead to ischemia and hypoxia. Therefore, early detection, early diagnosis and early treatment of TCS are advocated to avoid neurological damage or to create conditions for recovery of already damaged neurological function and to avoid new or persistent neurological damage. The common symptoms of TCS are: pain, which is the most common symptom and is indescribable, but without segmental distribution; dyskinesia, which is mainly progressive lower limb weakness or difficulty walking, usually bilateral or unilateral, and later may gradually develop muscle atrophy, horseshoe inversion, etc. The lumbosacral region can have sensory disorders, mainly hypoesthesia; bladder and rectal dysfunction, mainly manifested as urine loss and incontinence; skin abnormalities in the lumbosacral region: some pediatric patients have spondylolisthesis, skin sinus tracts, hirsutism, localized hemangioma or subcutaneous masses (less than 40%). The child’s growth and development, sudden pulling movements, spinal stenosis, and trauma can suddenly exacerbate symptoms. Raising awareness of TCS, avoiding missed diagnoses and misdiagnoses, and timely management to prevent delays are of great prognostic importance. Surgery is the only means of treating TCS. Surgery should be performed as soon as the patient’s general condition allows. Although the need for prophylactic surgery is still debated, most people now advocate that it is necessary to perform prophylactic surgery to minimize neurological damage. Because the entire pathophysiological process of TCS is not fully understood, it is impossible to determine and predict when neurological impairment will occur, and once it does occur, it is often irreversible. The goals of surgery are: to remove the cause of TCS and completely release the spinal cord embolus; to maximize neurological protection, especially of the distracted conus and corresponding spinal nerves; and to correct the accompanying spinal deformity. After surgery, the spinal cord is relieved of the strain, the spinal cord, especially the conus and the corresponding spinal nerves, is restored to normal local blood supply, and nerve function can be gradually restored. The current surgical approach is a significant improvement over that of the 1980s, and microsurgical techniques and intraoperative electrophysiological monitoring of neurological function have gradually become the gold standard for TCS surgery. Microsurgical techniques allow for careful isolation and resection of the spinal cord tethering, maximizing the spinal nerves that are closely related to the tethering to preserve maximum neurological function. Intraoperative electrophysiological monitoring of spinal cord and spinal nerve function can be monitored in real time, which not only improves the safety of surgery, but also provides real-time information on whether the spinal cord embolus has been completely released. It is especially suitable for complex cases, such as mixed fatty spinal bulge and TCS without release of the embolus in the first surgery, which can greatly improve the success rate of surgery. If TCS is left untreated, its symptoms tend to worsen progressively within a certain period of time. The rate and degree of improvement depend on a number of factors, including the cause and course of TCS, whether and to what extent neurological function was impaired before surgery, the surgical operation (whether the spinal cord embolus was completely released during surgery, whether neurological function was well protected, and whether the associated deformity was corrected), and the postoperative care and rehabilitation.