Study Design. Treatment of congenital kyphosis in myelomeningocele is a difficult problem. Current thinking supports kyphectomy and postoperative internal fixation. Objectives. Since 1989, vertebral resection with modified Luque fixation has been the procedure of choice for correction of myelomeningocele kyphotic deformity at the author's institution. The study objective was to evaluate long-term results with this technique. Summary of Background Data. Most investigators agree that kyphotic deformity in myelomeningocele should be treated with vertebral resection. There is less uniform consensus about postoperative fixation. Reports in the literature support fixation with modified segmental instrumentation. Methods. Sixteen patients, observed for an average of 57.2 months (range, 36-94 months), underwent vertebral resection from the proximal aspect of the apical vertebra cephalad into the compensatory lordotic curve. Fixation was segmental instrumentation wired to the thoracic spine and anterior to the sacrum. Results. The average blood loss was 1121 mt (range, 450-2580 mt). Kyphotic deformity averaged 111 degrees before surgery (range, 75-157 degrees), 15 degrees after surgery (range, -18-36 degrees) and 20 degrees at latest follow-up (range, -17-83 degrees). Loss of correction was 60 (range, 0-27 degrees). Postoperative immobilization was with a thoracolumbosacral orthosis for 18 months. Complications occurring in 8 of the 16 patients were transient headache, superficial wound breakdown, supracondylar femur fractures, and one late infection secondary to skin breakdown that necessitated early rod removal, resulting in some loss of correction. Conclusions. Kyphectomy is an excellent method of correcting rigid kyphotic deformity in the patient with myelodysplasia. Segmental spinal instrumentation provides three distinct advantages: rigidity of the construct, greater correction of the deformity and low-profile instrumentation.