Background <p>Retinoblastoma (RB) is the most common childhood intraocular malignancy, with substantial global disparities in outcomes. This study aimed to provide pooled estimates of incidence, survival, mortality, and metastasis based on population-based data.</p> Methods <p>A systematic review and meta-analysis was conducted in PubMed, Scopus, Web of Science, Embase, and the Cochrane Library through August 2025. Eligible studies were population- or registry-based and reported at least one epidemiological outcome. Random-effects models were applied, with assessments of heterogeneity, publication bias, and subgroup analyses.</p> Results <p>Twenty-five studies (52 study arms) were included. The global pooled incidence of RB was 0.12 per 1,000,000 (95% CI: 0.09–0.14), with high heterogeneity (I² = 95%). Incidence was highest in the Americas and Europe and lowest in Africa. One-, three-, and five-year survival rates were 94% (95% CI: 90–98), 81% (95% CI: 74–89), and 91% (95% CI: 87–94), respectively. Pooled mortality was 13% (95% CI: 9–16), and mortality increased with older age at diagnosis. The pooled metastasis rate was 6% (95% CI: 2–9).</p> Conclusions <p>RB incidence and survival show striking geographic and socioeconomic disparities. Delayed diagnosis and limited access to care drive persistently poor outcomes in many low-resource regions, underscoring the need for improved registries, earlier detection, and equitable treatment worldwide.</p>