Retinoblastoma (RB), the most common primary intraocular malignancy in infants and young children, poses a serious threat to visual function and the life of children when systemic metastasis occurs. Circular RNA (circRNA) is a recently discovered class of non-coding RNA that mainly functions as competitive endogenous RNA by regulating gene expression through competing with microRNA. circRNA can function as oncogenes or tumor suppressors, regulating various biological processes in RB cells, such as proliferation, migration, apoptosis, autophagy, and drug resistance, thereby providing new therapeutic targets for RB. In addition, the differential expression of circRNA in tumor tissues or exosomes is expected to be a potential biomarker for RB. Further studies and explorations are still needed for the functions and regulatory mechanisms of circRNA in RB to reveal their precise roles in organisms and potential clinical applications.