AIM: To report the characteristics and clinical presentations of retinoblastoma in a series of pediatric patients from Iran. METHODS: In this retrospective study, profiles of pediatric patients with retinoblastoma archived in a referral eye hospital in north-west of Iran during 7 years (n=40 patients with 57 eyes) were reviewed. Demographics, as well as the laterality, clinical manifestations and the types of treatment were the major endpoints. The Student’s t test, Mann-Whitney U-test, Chi-square or Fishers’ Exact test was used for analysis where appropriate. RESULTS: There were 23 cases (57.7%) with unilateral and 17 cases (42.5%) with bilateral involvement. The male to female ratio was 1 to 1.4 with a mean admitting age of 24.0±11.3 (range: 5-62) months. The mean diagnosis delay was 7.4±9.6 months (range: 10 days to 13 months). The most common presenting sign was leukocoria (97.5%) followed by proptosis (7.5%), strabismus (7.5%), hyphema (5%), orbital cellulitis (5%) and glaucoma (2.5%). Enucleation was performed in 95.7% of the cases with unilateral involvement and at least one eye of the patients with bilateral disease. A second enucleation was performed in other 3 patients (17.6%) of the latter group. CONCLUSION: This is the first study evaluating a series of Iranian children with retinoblastoma.