The literature was systematically reviewed to determine if children with spina bifida have lower self-concept compared with their peers with typical development. Relevant trials were identified by searching electronic databases, supplemented by citation tracking. Of 803 papers initially identified, 15 met the inclusion criteria. Meta-analysis revealed children with spina bifida scored significantly lower than children with typical development for the domains of global self-worth (d=-0.39, 95% confidence interval [CI] -0.65 to -0.12); physical appearance (d=-0.26, 95% CI -0.46 to -0.06); athletic competence (d=-0.45, 95% CI -0.67 to -0.22); social acceptance (d=-0.33, 95% CI -0.55 to -0.11); and scholastic competence (d=-0.43, 95% CI -0.66 to -0.21). There was no difference between the groups for the behavioural conduct domain. Children with spina bifida on average have a lower self-concept than their peers with typical development. Clinicians need to take account of this information in planning the assessment and treatment of this group.