There are few areas within the field of pediatric orthopaedics that have had more profound advancements in the past 15 years than the care of children with early onset scoliosis. Although the number of underlying diagnoses and associated comorbidities are numerous, there are several essential underlying principles: comprehensive initial evaluation, close and ongoing communication with caregivers, exhausting the nonoperative methods before moving to surgery, diligent informed consent, preparing the family for potential complications and unplanned surgery, and a thoughtful approach to the end of lengthening or expansion, with final fusion surgery in most cases. This review aims to present the state-of-the-art in the care of early onset scoliosis, including evaluation, operative decision-making, and management of complications. Although the care is complex, and problems are common, many children successfully treated would not have survived into adulthood without the modern methods available to manage their severe spinal and chest wall deformities. Advances on the horizon, including new imaging modalities, the possibility of nonoperative lengthening, and increasingly sophisticated final fusion procedures, offer even more hope for the next generation of children with early onset scoliosis.