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2 Background/aims: Retinoblastoma is a highly malignant eye tumor in children with different survival rates across the world. The aims of this study are to determine the globe and patient survival in children with retinoblastoma in a major referral center in Iran. Methods: 156 eyes of 105 consecutive patients with retinoblastoma were enrolled from 2001 to 2007. All demographic data, family history, presenting symptoms, duration of symptoms, ocular findings and treatment modalities that were used for the patients were collected. For patient survival, event was defined as death and for globe survival as enucleation. Results: Mean age at diagnosis was 28.5 months (unilateral, 27.4 months; bilateral 30 months). Five patients had positive family history. Fifty two percent of the cases were unilateral and 48% of them were bilateral. The most common presenting sign was leukocoria (64.8 %) followed by strabismus (28.2%). Enucleation was done primarily for 75.9 % of unilateral cases an