AIM Scoliosis is a frequently occurring and serious complication of severe cerebral palsy (CP).This systematic review aims to the assess the risk factors associated with the emergence and progression of scoliosis in children with CP functioning at level IV or V of the Gross Motor Function Classification System (GMFCS).METHOD Relevant studies conducted from 1966 to March 2009 were identified in Embase, Medline, and Scopus. The methodological quality of the studies was assessed and relations between risk factors and scoliosis were documented systematically.RESULTS Ten studies were included. They had a low level of evidence and heterogeneous methodological quality. No systematic associations were found between type of CP and scoliosis or between age, type, and location of scoliotic curve and progression of scoliosis. Weak evidence was present for an association between the severity of CP, hip dislocation, and pelvic obliquity and scoliosis.