Congenital segmental spinal dysgenesis is a rare lesion that usually is associated with neurological deficits. Affected infants who are neurologically intact may suffer progressive kyphosis and subsequent neurological deterioration. Results of external casting, laminectomy with spinal fixation, or bone grafting of the defect have been disappointing. The authors present a successful staged surgical management scheme consisting of complete resection of the dysraphic vertebral bodies, maintenance of normal vertebral distance with rib strut grafting, and fusion with autogenous bone.