The treatment of spinal deformities in children with myelomeningocele poses a formidable task. Multiple medical comorbidities, such as insensate skin and chronic urinary tract infection, make care of the spine difficult. A thorough understanding of the natural history of these deformities is mandatory for appropriate treatment to be rendered. A team approach that includes physicians from multiple specialties provides the best care for these patients. The two most challenging problems are paralytic scoliosis and rigid lumbar kyphosis. The precise indications for surgical intervention are multifactorial, and the proposed benefits must be weighed against the potential risks. Newer spinal constructs now allow for fixation of the spine in areas previously difficult to instrument. Complications appear to be decreasing with improved understanding of the pathophysiology associated with myelomeningocele.