Jarcho-Levin syndrome (JLS) is a rare congenital dysostosis characterized by multiple vertebral and costal anomalies. The combination of JLS and neural tube defect is rare. Only six cases of JLS accompanying diastematomyelia have been reported; all were in infants or children. We present the case of a 37-year-old female patient with JLS who also had diastematomyelia in lumbar vertebral level. This is the seventh case of JLS with diastematomyelia, and the first adult case.