Skeletal dysplasias are a diverse group of conditions that affect cartilage and bone development. Although these conditions are rare, an estimated incidence of 2.3–7.6 per 10,000 population, spinal involvement is common. It is important to understand the developmental anatomy of the spine in the context of each skeletal dysplasia for effective management. Spinal problems observed include cervical instability, sagittal and coronal deformity, and spinal stenosis. Some deformities are transient: thoracolumbar kyphosis in achondroplasia, which typically resolves at the onset of ambulation, and cervical kyphosis in diastrophic dysplasia. Cervical instability and stenosis can be progressive and result in neurological injury. The management of progressive spinal deformity in children with skeletal dysplasias can be very challenging. The osseous elements are small and fragile, and bone formation is limited. Many of these children have short trunks and restrictive lung disease. The aim is to preserve spinal growth and manage the deformity. These children can have severe musculoskeletal problems but often the most life-threatening problems are in the extra-musculoskeletal systems and include cardiac, respiratory, immunological, and gastrointestinal issues. These conditions must be optimized to ensure a successful outcome. A coordinated, multidisciplinary team is essential for clinical effectiveness.