Background <p>Recent studies indicate parents of childhood cancer survivors experience high psychological burden. However, there is limited knowledge regarding the mental health of parents of children with retinoblastoma (RB) in China. Thus, the purpose of this study was to assess anxiety, depression, fear of disease progression (FoP), and family function among parents of children with RB, and to analyze associated factors.</p> Methods <p>This cross-sectional study enrolled 151 RB patients and 185 of their parents. Parents completed the Beck Anxiety Inventory (BAI), Beck Depression Inventory (BDI), Fear of Progression Questionnaire-Short Form/Parent version (FoP-Q-SF/PR), and Family APGAR Index to assess the levels of anxiety, depression, FoP, family function. Univariate and multivariate analyses were performed to identify associated factors.</p> Results <p>Clinically relevant anxiety and depression were reported in 34% and 41% of parents of children with RB. FoP-related dysfunction was observed in 68.1% of parents. More than half of the families (54.9%) exhibited family dysfunction. Anxiety level demonstrated positive correlations with urban residency and BDI scores. The increase in the number of intravenous chemotherapy (IVC), tumor recurrence, urban residency and BAI scores were associated with parental depression levels. Additionally, multivariate analysis included IVC, religious belief, ethnic minority groups and BDI scores as independent risk factors for FoP.</p> Conclusions <p>Anxiety, depression and FoP represented substantial burdens for parents of children with RB, which were relate to IVC, age at diagnosis, tumor recurrence, place of residence, and education level of parents. Consequently, during treatment of RB, particular attention needs to be paid to the psychological health of parents, psychological interventions should be provided when necessary.</p>