b＞Introduction : /b＞Jarcho-Levin syndrome, first described in 1938, is a rare disorder. It was characterized by a short neck, a short and constricted thorax leading to respiratory insufficiency. Solomon classified Jarcho-Levin syndrome into two subtypes, namely spondylothoracic dysostosis and spondylocostal dysostosis, based on survival rate, inheritance pattern and the extent of skeletal anomalies. Spondylocostal dysplasia, an autosomal dominant and recessive disorder, had intrinsic rib anomalies like bifurcation and broadening. We report spondylocostal dysplasia form of Jarcho-Levin syndrome. b＞Case report : /b＞ The neonate weighing 3164g was born by Cesarean section at 37 gestational weeks. Apgar scores were 7 at one minute and 9 at five minute. The newborn examination showed a short neck, accessory nipples on the left side and a single umbilical artery. A spine CT showed multiple vertebral deformities with hemivertebrae in Rt T1, 2, 10 and fusion anomaly in T6~7 with lateral wedging and scoliosis in T-spine convexity to the right. A chest and abdomen CT showed right sides cardiac deviation, partial left rib agenesis, hypoplastic left thorax and left renal agenesis. Echocardiography showed no anomalies except patent ductus arteriosis( PDA). She was treated with ibuprofen to close a PDA. After about 2 hours after birth, she had chest retraction and respiratory difficulty requiring mechanical ventilation. At hospital day 16, she had been extubated. She was able to take feedings very well and discharged at hospital day 28. Chromosome studies using GTG-banding gave normal results (46,XX). She had no mutations in the DLL3 gene. Until 1 month after discharge, she grew well without pneumonia and pulmonary insufficiency. b＞Conclusion : /b＞We report a case of Jarcho-Levin syndrome with left renal agenesis.