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Objective Children born extremely preterm (EPT) vary greatly regarding academic outcomes, underscoring the need to investigate subgroups in large representative samples. This study aimed to (1) examine academic outcomes in EPT children versus full-term controls and (2) examine the role of maternal education, child sex, maternal origin, cognitive ability and the presence of attention deficit hyperactivity disorder (ADHD) or autism spectrum disorder (ASD) diagnosis.Design National cohort study.Setting Sweden.Patients 12-year follow-up of a cohort study, the Extremely Preterm Infants in Sweden Study, including children born EPT (n=449) and full-term controls (n=365).Outcome measures Data on school grades (mathematics, Swedish and English) and special education school attendance were obtained from registers.Exposure EPT group versus controls and subgroup analyses within the EPT group.Results Compared with full-term controls, children born EPT more often attended special education schools (13.6% vs 0.5%; OR 28.5 (95% CI 6.9 to 117.5)) and those attending mainstream school more often received at least one failing grade (32.3% vs 5.2%; OR 8.7 (95% CI 5.2 to 14.5)). Of children with gestational age (GA) ≤23 weeks, 39% attended mainstream school and passed all three core subjects, compared with 65% of those born at 26 weeks (OR 0.34 (95% CI 0.17 to 0.62)). Among EPT children attending mainstream education, low GA, low maternal education, cognitive impairment and ADHD/ASD diagnosis increased the risk of poor academic achievement.Conclusions EPT birth was associated with poor academic achievement, but the risk varies substantially within the EPT group, primarily based on cognitive impairment, ADHD/ASD diagnosis and maternal education. However, the risk of poor academic achievement among children born EPT remained after adjusting for confounders and mediators.