Introduction. Tethered cord syndrome (TCS) is a progressive damage of spinal cord and occurs when the distal spinal cord is adherent to inelastic tissue in close proximity to it. The pathology is often associated with spinal disraphism – spina bifida occulta, myelomeningocoele, intraspinal lipoma. The essential problem in tethering is a progressive neurodegenerative disorder of the spinal cord caused by permanent traction on the caudal end during the trunk movements. This local lesion occurs with several symptoms like sensory and motor deficits affecting the lower extremities, bowel and bladder dysfunction, lumbar-sacral area and legs pain, and muscoskeletal deformities. The purpose of the study was to assess results of surgical treatment of tethered cord in children and an attempt to define when a surgical treatment in children with tethered cord syndrome should be performed? Methods and clinical material. The retrospective analysis of 21 cases with tethered cord syndrome in children treated in Neurosurgery Department Children’s University Hospital in Krakow, was conducted. In patients who underwent spinal cord untethering, we reviewed basic disease, common symptoms of TCS, associating neurological disturbances, level of tethering, results of the therapy and possible complications. Results. All patients with complaints of pain and neurological disturbances were qualified for surgical treatment of tethered cord. The improvement was stronger pronounced in lower limbs than in sphincters. The associated back pain and lower limbs responds best to surgical treatment. In two patients was noticed bladder dysfunction and sensory and motor deficits in lower limbs. Conclusions. Surgical untethering should be performed in patients with progressive neurological deficits before serious neurological dysfunction occurs. Early detection and intervention offers the patient the best chance for a good outcome. In cases when pathology is diagnosed accidentally with no symptoms, decision whether to operate on children with tethered cord could be based on the level of tethering. When it is below L2 decision about surgical release should be considered. In cases with tethering below L4 surgical treatment should be performed to avoid in the future symptoms of spinal roots and spinal cord damage or Chiari malformation II.