From 1985 to 1997, 442 children were treated for syringomyelia. One hundred and eighty eight had syringomyelia in association with Chiari I malformation. In 65% of the case scoliosis was the initial symptom. All these children were treated by posterior fossa decompression, 95% were clinically improved or stabilized. The scoliosis remained unchanged or improved in only 44% of the cases. Many other causes of craniovertebral junction anomalies have been found (Chiari II, achondroplasia, mucopolysaccharidosis, craniosynostosis, Dandy-Walker malformation, posterior fossa cysts, birth injuries, or other causes of raised intracranial pressure). All these children were treated by posterior fossa decompression or when possible treatment of the cause. Features and results are presented. Spinal causes (diastematomyelias, lumbar lipomas, ventricule terminalis, spinal tumors ...) are presented. Whe discuss the treatment of scoliosis with specific attention to scoliosis associated to isolated syringomyelia.