Survival of retinoblastoma is >90% in developed countries but there are significant differences with developing countries in stage at presentation, available treatment options, family compliance, and survival. In low‐income countries (LICs), children present with advanced disease, and the reasons are socioeconomic and cultural. In middle‐income countries (MICs), survival rates are better (>70%), but there is a high prevalence of microscopically disseminated extraocular disease. Programs for eye preservation have been developed, but toxicity‐related mortality is higher. Although effective treatment of microscopically extraocular disease improved the outcome, worldwide survival will be increased only by earlier diagnosis and better treatment adherence. Pediatr Blood Cancer 2011;56:341–348. © 2010 Wiley‐Liss, Inc.