Spondylocostal dysostosis is one of two phenotypic groups of Jarcho-Levin syndrome, which characterized by short trunk dwarfism associated with rib and vertebral abnormalities. Here, we report a case of a newborn infant with spondylocostal dysostosis, which illustrates the typical findings of the syndrome. The early diagnosis in prenatal and neonatal period allow to provide appropriate genetic counseling and managing.