The diagnostic performance of ACE in pediatric sarcoid uveitis was found to be poor. NPV exceeded 90% but was based on a significant number of false negatives, indicating a high risk of misdiagnosis. Likelihood ratios confirmed the limited diagnostic value of ACE. Considering age groups and clinical manifestations may enhance precision but requires larger studies. Serum ACE used as a diagnostic marker in pediatric sarcoid uveitis warrants caution and should be interpreted alongside other factors.