BACKGROUND: The relationship between Kawasaki disease (KD) and neurodevelopmental disorders (NDDs) remains unclear. This study aims to explore the association between them. METHOD: A systematic review was conducted using PubMed and Embase databases from inception to May 1, 2024 (INPLASY202450027). We included case-control or cohort studies comparing KD patients to healthy controls in assessing attention-deficit/hyperactivity disorder (ADHD), autism spectrum disorder (ASD), and intellectual disability (ID). The meta-analysis employed a random-effects model to calculate effect sizes using hazard ratios (HRs) with 95% confidence intervals (CIs) for the disease occurrence. Moreover, mean differences (MDs) were used to calculate intelligence quotient (IQ). RESULTS: Four eligible studies, including 1,454,499 participants, were analyzed for ADHD, ASD, and ID. The risk of ADHD in KD patients was higher than in healthy controls (HR[95%CI] = 1.76[1.21-2.57]). However, the risks of ASD (HR[95%CI] = 1.68[0.47-5.94]) and ID (HR[95%CI] = 1.39[0.52-2.63]) were not significantly different between KD and controls. Additionally, three studies with 365 participants were analyzed for IQ. IQ comparisons showed no significant differences in full IQ (MD[95%CI]=-0.01[-2.44-2.42]), verbal IQ (MD[95%CI]=-1.05[-4.42-2.33]), and performance IQ (MD[95%CI]=-0.08[-2.75-2.59]). CONCLUSION: This study indicates that individuals with KD have a higher risk for ADHD but not for ASD or ID. TRIAL REGISTRATION: INPLASY, INPLASY202450027. Registered 05 May 2024, https://inplasy.com/inplasy-2024-5-0027/ .