Objectives: To longitudinally evaluate self-reported and proxy-reported health status (HS) and quality of life (QoL) of school-aged children born with esophageal atresia (EA). Methods: We obtained Pediatric Quality of Life Inventory (HS) and DUX-25 (QoL) questionnaires from children born with EA between 1999 and 2011 at 8 and/or 12 years old. Children completed self-reports during neuropsychological assessments in a prospective longitudinal follow-up program. Parents filled out proxy-reports at home. Total and subscale scores were evaluated longitudinally and compared with sex-specific reference norms. Results: In total, 110 participants (62% boys) were included. Self-reported HS improved significantly between 8 and 12 years for both boys (mean difference [md] 4.35, effect size [ES] 0.54, P ¼ 0.009) and girls (md 3.26, ES 0.63, P ¼ 0.004). Proxy-reported HS tended to improve over time, while self-reported and proxy-reported QoL tended to decline. Self-reported HS at 8 years was below normal for both boys (md À5.44, ES À0.35, P < 0.001) and girls (md À7.61, ES À0.32, P < 0.001). Girls' self-reported QoL was below normal at 8 (md À5.00, ES À0.18, P ¼ 0.019) and 12 years (md À10.50, ES À0.26, P ¼ 0.001). Parents reported normal HS at both ages, whereas they rated the QoL of their daughters below normal at 12 years (md À10.00, ES À0.16, P ¼ 0.022). All above results are total scores. Conclusions: Self-reported and proxy-reported HS of children with EA improved between 8 and 12 years, while their QoL tended to decline. We recommend to consider HS and QoL as two separate concepts and to measure both simultaneously and longitudinally when evaluating the burden of disease.