Summary:The tethered cord syndrome (TCS) has been diagnosed with increasing frequency as our understanding of the disease has progressed. In addition, modern neuroimaging and neurosurgical techniques have advanced to the point where prompt diagnosis and treatment have become the norm. The vast majority of patients with a tethered spinal cord, if left untreated, will develop progressive neurological dysfunction characterized by bowel and bladder problems and lower extremity weakness, often associated with back and leg pain usually described as burning and dysesthetic in nature. At times progressive scoliosis is seen. Once neurological dysfunction has occurred, there is only a moderate chance of improvement with surgery. Consequently, current recommendations are that appropriate neuroimaging studies of the entire spine be done at the time of initial diagnosis to rule out second lesions. Surgical treatment should not be delayed unless the risk of resection of a complex lesion outweighs the benefit to the patient. Newborns should be operated on early in life. This approach to treatment seems to provide optimal outcome.