Paraplegia or paraparesis is uncommon in patients with neurofibromatous scoliosis. The main causes of spinal cord compression in neurofibromatosis are vertebral angulation, vertebral subluxation, and tumorous lesions around the spinal cord. We report a rare case of paraparesis due to spinal cord compression by a rib penetrating the spinal canal in a patient with neurofibromatous scoliosis. There was complete recovery after laminectomy and proximal resection of the compressing rib along with combined anterior and posterior spinal fusion.