Tethered cord syndrome (TCS) is a diverse clinical entity characterized by symptoms and signs which are caused by excessive tension on the spinal cord. The majority of cases are related to spinal dysraphism. TCS can present in any age group, and presentations differ according to the underlying pathologic condition and age, with pain, cutaneous signs, orthopedic deformities and neurological deficits being the most common. Surgical untethering is indicated in patients with progressive or new onset symptomatology attributable to TCS. The surgical strategy aims to release the tethering structure and thus the chronic tension on the cord. Early operative intervention is associated with improved outcomes. Pain relief is accomplished in almost all cases. Realistic surgical goals include relief of pain and stabilization of neurological function, although improvement in function is often seen. Cord untethering can also halt the progression of scoliosis. The benefits of surgery are debated in asymptomatic patients and patients with normal imaging.