AIM: To evaluate clinical characteristics, treatment patterns and long-term quality of life (QoL) among children with retinoblastoma (RB) managed at a single tertiary center in China. METHODS: Totally 62 consecutive patients (71 eyes) diagnosed with RB were retrospectively reviewed. The mean age at first visit was 22.13±17.87mo; 35 (56.45%) were male. Unilateral disease occurred in 53 patients (85.48%) and bilateral disease in 9 (14.52%). According to the international intraocular retinoblastoma classification (IIRC), eyes were staged as A (n=6), B (n=9), C (n=9), D (n=21), E (n=19), and extraocular (n=7). Treatments followed stage-based indications. QoL at follow-up was assessed using validated pediatric ophthalmic instruments completed by patients and/or parents. RESULTS: The mean follow-up duration was 42.9±6.49mo. Overall survival was 90.32% (56/62), mortality was 9.68% (6/62). The overall globe-preservation rate was 64.79% (46/71), and 71.88% (46/64) for intraocular RB. Eye salvage by IIRC stage was 100% for A–C, 71.43% (15/21) for D, and 36.84% (7/19) for E; no eyes were preserved in extraocular disease. Compared with the globe-preservation group, enucleated children had significantly lower scores in appearance, social functioning, and role domains on QoL measures (P<0.05). Across all PedEyeQ domains, children with preserved globes scored higher than those who underwent enucleation (P<0.05). CONCLUSION: Most children in this cohort present with intermediate-to-advanced disease, which limit eye-preservation opportunities. While survival approached contemporary benchmarks, QoL deficits are most pronounced in appearance, social participation, and role functioning after enucleation. Additionally, parents of children who underwent enucleation often experience heightened anxiety about their child’s vision and social integration. These findings underscore the need for earlier detection and integrated psychosocial support alongside stage-appropriate therapy.