Nutritional, environmental factors and genetic disorders are the main factors that play role in the development of neural tube defects (NTDs). In the spinal surgery, the most common congenital lesions presenting to medical attention are the diverse forms of the spinal dysraphism and caudal spinal anomalies. This report describes a rare case of congenital defective thecal sac that terminated in the skin at L3-4 level of the child who underwent surgery to repair meningomyelocele in our institute when he was 11- day old. After the urodynamic study was performed the surgery to repair swelling at L3-4 level had done. The child followed-up for four years from his birth. The child is doing well, without growth or mental retardation. Herein, the authors describe the management of multiple congenital malformations in children.

Keywords: Defective congenital thecal sac, laminoplasty, spina bifida occulta, intraoperative neurophysiological monitoring, urodynamic study