In Brief:
Three-dimensional computed tomography scanning was undertaken, to improve our understanding of the spine maldevelopment in a pair of male siblings with spondylocostal dysostosis. Standard radiographic documentations were noncontributory. We observed extensive fusion of the skull base with the entire spine, resulting in a remarkable development of solid, immobile, and fixed bony ankylosis of serious outcome. To the best of our knowledge, no previous computed tomography findings of the spine has been published, in patients with spondylocostal dysostosis.