**Abstract**

Retinoblastoma (Rb) is the most common primary intraocular malignancy of childhood. The incidence of Rb is stable worldwide at one case per 16,000–18,000 live births. It is estimated that 7800–8800 Rb cases were newly diagnosed globally in 2017. Over 80% of these are in low- and middle-income countries (LMICs) in Asia and Africa. So far, there is no validated evidence that retinoblastoma incidence is associated with gender, ethnicity or geographical factors. A link between human papillomavirus (HPV) and Rb is being investigated to establish its role in the pathophysiology of the sporadic form of the disease. Survival rates for Rb vary greatly between countries: while almost all Rb cases from high-income countries survive, cases in LMICs have a mortality rate of up to 70%.

**Keywords:** retinoblastoma, incidence, *RB1*, hereditary
