Despite being a rare pediatric cancer arising in the developing retina from red/green cone precursors, retinoblastoma is the most common eye cancer worldwide and occupies an emblematic position in oncology and human genetics for the following reasons:-Historically, the discovery of <i>RB1</i> and the recessive nature of its mutations led to the prototypic description of anti-oncogenes or tumor suppressor genes [...]. Here, RB1 is linked to retinoblastoma.