Aim To describe the clinical presentation and group classification of newly diagnosed retinoblastoma patients in China, and to identify future priorities for improving the medical care of this disease. Methods The retrospective review included all patients who presented with previously untreated intraocular retinoblastoma at Beijing Tongren Hospital between August 2006 and August 2009. The clinical charts were reviewed and fundus images taken for all patients were analysed. The tumours were classified according to the International Intraocular Retinoblastoma Classification. Results The study included 595 eyes (470 patients) with untreated retinoblastoma. The mean age at diagnosis was 23 months, with 84% of the patients being younger than 3 years at the time of diagnosis. Bilateral retinoblastoma was present in 150 (32%) of the patients. Bilateral retinoblastoma as compared with unilateral retinoblastoma was diagnosed at a significantly (p<0.001) younger mean age (15 months vs 27 months). Leucocoria was the most common leading sign (73% of patients). The vast majority of eyes (501/595 or 84%) presented with an advanced stage of the tumour (Group D or E of International Intraocular Retinoblastoma Classification). Conclusion In China, diagnosis of retinoblastoma occurs at a relatively advanced clinical stage of the disease. An intensified programme for earlier detection of retinoblastoma including educating parents and physicians may be warranted to improve clinical outcome with fewer enucleations.