Tethered cord syndrome, seen in patients with spinal dysraphism, is a progressive neurological deterioration due to stretching of the spinal cord. Spinal dysraphism results from defects during embryonic closure of the neural tube, usually in the lumbosacral region. In tethered cord syndrome an abnormally low conus medullaris is tethered by intradural abnormalities such as a short, thickened filum terminale, fibrous bands, a lipoma or diastematomyelia. This article distinguishes tethered cord syndrome from other forms of occult spinal dysraphism and describes and compares normal anatomy to pathological changes. Clinical symptoms and nursing management of the pediatric patient with tethered cord syndrome will be exemplified by a case study presentation.