Abstract: Congenital scoliosis is a failure of vertebral formation，segmentation，or a combination of the 2 arising from abnormal vertebral development.Hemivertebral deformity caused by the former is the main cause of congenital scoliosis.Its etiology is complex and may be related to genetic and environmental factors.The diagnosis depends on clinical manifestations and imaging findings.Currently，imaging examinations include fetal spinal ultrasound and MRI，postnatal spinal ultrasound in infants，whole spine X-rays，CT，and MRI.At the same time，the genitourinary and cardiac ultrasound should be performed.Conservative treatment includes plaster，brace，the therapeutic effect is not good enough to completely correct deformity，so early surgical treatment is needed.The operative methods include in situ fusion，convex epiphysis arrest，hemivertebral resection，spinal fusion and spinal non-fusion.

Key words: congenital scoliosis, etiology, diagnosis, treatment