Kyphoscoliosis (KS) is a significant clinical problem with no precise guidelines for management, especially concerning respiratory pathology. No exhaustive systematic review has yet been performed. The aim was to conduct a systematic review of available data concerning the pathophysiology and treatment of kyphoscoliosis. An electronic systematic search compliant with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was conducted. The Pubmed database was examined and the search was updated to November 10, 2016. In total, 188 articles were screened, and 52 full text articles were then assessed for eligibility. As 24 of them met exclusion criteria, only 28 articles were further analysed. These articles were published in 18 journals from 1959 to 2015, with 25 being original studies, and one randomised control trial. The total corpus included 18 prospective studies, 7 retrospective studies and 3 case reports. KS is a significant complex, multidisciplinary clinical problem. The heterogeneous nature of the majority of published studies prevents unequivocal conclusions being drawn. Despite a great progress in knowledge about the respiratory system functioning and pathology in KS, the treatment seems to be not yet quite satisfactory. Therefore, there is a strong need for large prospective studies and unified clinical guidelines on the management of this group of patients.