ABSTRACT
Jarcho-Levin syndrome is a congenital disorder characterized by abnormal vertebral segmenta-tion, defects affecting spine formation with complete bilateral fusion of the ribs at the costover-tebral junction producing a “crab-like” configuration of the thorax. This syndrome is composed of two subgroups: spondylothorasic and spondylocostal dysostosis. While vertebral deformities are frequent in spondylothorasic type, costal abnormalities are common in spondylocostal type. Jarcho-Levin syndrome may be associated with neural tube defects, cardiac, renal, and gastroin-testinal anomalies. In this report, a newborn diagnosed to be Spondylothorasic type Jarcho -Levin syndrome and associated with meningomyelocele and hydrocephalus was presented with litera-ture highlight because of its rarity.