Cervical spine instability in children is rare but not exceptional and may be due to many factors. Although it mostly occurs at the upper cervical spine, all vertebrae from the occiput to T1 may be involved. It may be acute or chronic, occurring secondary to trauma or due to congenital anomaly, skeletal or metabolic dystrophy or rheumatoid arthritis. It can be isolated or associated with other musculoskeletal or visceral anomalies. A thorough knowledge of embryology, anatomy, physiology and physiopathology of the cervical spine in children is essential to avoid pitfalls, recognize normal variants and identify children at risk of developing cervical spine instability and undertake the appropriate treatment.