Severe spinal deformity in progressive neuromuscular disease (NMD) leads to multiple problems, including poor sitting balance, difficulty with upright seating and positioning, pain, difficulty in attendant care, and potential exacerbation of underlying restrictive respiratory compromise. Severe scoliosis and pelvic obliquity can in some instances completely preclude upright sitting in a wheelchair. This article reviews the prevalence, natural history, and management of scoliosis in neuromuscular diseases at greatest risk for progressive spinal deformity.