Pediatric spinal deformity is a common problem facing the pediatrician and orthopedic surgeon. Most commonly seen is idiopathic scoliosis. Early diagnosis and treatment are important. Although the etiology of idiopathic scoliosis remains unclear, there continues to be a search for genetic markers and studies for the modes of inheritance. Idiopathic scoliosis is more clearly understood recently and is recognized as a complex three-dimensional deformity. Prognostic indicators for juvenile scoliosis have been identified. Surgical management of idiopathic scoliosis continues to evolve, and now a thoracoscopic endoscopic technique is available. Long-term follow-up has demonstrated generally satisfactory results with spinal fusion surgery. Other syndromes such as Klippel-Feil, familial dysautonomia, and Marfan syndrome demonstrate high rates of scoliotic deformities. Most are unresponsive to bracing and most often require surgical intervention. Although primary spinal neoplasms are uncommon, most are benign, and outcomes are generally satisfactory.