Children with cervical and craniocervical spine deformity can present with a variety of congenital or acquired conditions. Although it can be challenging, proper surgical treatment can improve or restore the child’s function and overall quality of life. Careful diagnosis and preoperative planning, along with a detailed knowledge of embryology and anatomy, are paramount for successful treatment. Future longitudinal studies and long-term health-related quality-of-life outcomes analyses are necessary in this select population of children.