Objectives Studies have reported the applications of F-18-dihydroxyphenylalanine (F-18-DOPA) PET in patients with congenital hyperinsulinism (CHI). The aim of this study was to systematically review and perform a meta-analysis of published data on the diagnostic role of F-18-DOPA PET in patients with CHI. Materials and methods A comprehensive computer literature search of studies on F-18-DOPA PET or PET/computed tomography (CT) in patients with CHI was conducted. The pooled sensitivity and specificity of F-18-DOPA PET or PET/CT in patients with CHI were calculated. The area under the receiver-operating characteristic curve was calculated to measure the accuracy of F-18-DOPA PET or PET/CT in patients with CHI. Results Ten studies comprising 181 patients with CHI were included in this meta-analysis. The pooled sensitivity of F-18-DOPA PET and PET/CT in detecting CHI was 88% on a per-patient-based analysis. The pooled specificity of F-18-DOPA PET and PET/CT in demonstrating CHI was 79%. The area under the receiver-operating characteristic curve was 0.92 on a per-patient-based analysis. Conclusion In patients with CHI, F-18-DOPA PET or PET/CT demonstrated high sensitivity and specificity. F-18-DOPA PET and PET/CT are accurate methods for the diagnosis of CHI. Nevertheless, possible sources of false-positive and false-negative results should be kept in mind. Nucl Med Commun 34: 347-353 (C) 2013 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.