Introduction: Recurrent tethered cord syndrome (TCS) is a neurosurgical condition that is associated with spinal dermoid cysts, myelomeningoceles and lipomyelomeningoceles most commonly. However, recurrent TCS due to myelomeningocele and neurofibromatosis type 1 (NF1) has not been reported.
Case Report: We describe a case of a 13-year-old boy with recurrent TCS and progressive spinal deformity with a severe kyphoscoliosis due to myelomeningocele and NF1. We also review the anterior and posterior surgical approaches to vertebrectomies along with their indications and contraindications. Finally, we also discuss specific risks of scoliosis repair in NF1 patients due to high probability of pseudoarthrosis.
Conclusion: We conclude that in patients with recurrent TCS, posterior fusion with shortening and without detethering was a successful procedure which should be considered as a primary surgical option. Anterior fusion may be delayed in such patients and used only if necessary given the additional risks in such medically fragile patients.

Keywords: Neurofibromatosis type 1, Pseudoarthrosis syringohydromyelia, Recurrent tethered cord syndrome, Vertebrectomy