To fill this gap, we report here the development of 2 Rb models generated through CRISPR/Cas9 inactivation of RB1 gene in hESC and derivation of patient-specific iPSC line from a child with heterozygous mutation (c.2082delC) in the RB1 gene, who developed bilateral malignant growths before the age of 4. Here, RB1 is linked to retinoblastoma.