Jarcho- Levin syndrome or costovertebral dysplasia or spondylocostal dysplasia or spondylocostal dystosis is a rare genetic disorder that represents a spectrum of short trunk skeletal dysplasia with variable involvement of the vertebrae and ribs1. Jarcho and Levin, in 1983 described a pattern of vertebral and costal anomalies distinctly different from the well known Klippel- feil syndrome2. The subtypes are spondylothoracic dysplasia predominant vertebral defects and spondylocostal dysplasia describes the variant with vertebral and instrinsic costal anomalies3. Despite being rare, this disorder has been focus in recent times due to controversies surrounding it4. The purpose of reporting this case is to bring clinical understanding of this rare disorder and review the emerging current knowledge about it. In this article, we report a case of spondylocostal dystosis in 2 month old female from Doda, Jammu.