Congenital vertebral synostosis (CVS) is a rare developmental condition due to failure of vertebral segmentation. Vertebrae and their intervertebral discs differentiate and resegment at the time of organogenesis during fetal life. Failure of this embryological process can result in the limitation of mobility of the involved segment. This inappropriate segmentation thus results in vertebral fusion or a block vertebra with subsequent vertebral synostosis. Long-term, such segmental fusion can increase osteoarthritis at levels below and above the fused segment due to excessive wear on these joints. Presentations can include congenital kyphosis and scoliosis. Patients may present with back and radicular pain, and possible myelopathy CVS usually occurs, in order of frequency, in the cervical, lumbar, and thoracic vertebral levels. This paper reviews congenital lumbar synostosis with associated findings and its clinical implications and embryological significance. A case illustration is also included.