Aims: Our aims are to investigate the extension of the unilateral retinoblastomaby the clinical, paraclinical and the anatomopathological means, at the moment of the first medic surgical intervention.Materials & methods: In this retrospective study conducted from 1989to 1999, for ten years, all patients with a unilateral retinoblastoma who have been operated at the Farabi Eye Hospital of Tehran have been investigated.A complete ocular examination under general anesthesia, sonography, CT Scan, bone marrow Cerebral spinal fluide (C.S.F) and then a complete anatomopathological examination has been conducted.Results: 139patients with unilateral retinoblastomahave been operated during these 10 years (enucleation, exoneration). 80 patients were males and 59 females. The mean-age of the pateints at the time of the intervention was 34.98±21.95 months (ranged between I month to 120 months). The duration of the first symptoms observed by the parents until the intervention was 5.24%6.96months (ranged between 2 days to 36 months). The first symptoms observed by the parents have been: leukocoria 79%, red eye 10.5% Strabismus 40.8%, exophthalmos 2.4%, buphthalmos 1.6%.At the fundi examination, in 97.1% of the cases more than half of the retina was occupied by the turnor (stage V of Reese-Ellsworth), in 11.5% of the eyes vitreous seedings was observed, 7.9% of the eyes had exophthalmos, 70.9% had buphthalmos, 4 patients had hypopion or iris metastasis, 2 patients had head and face metastasis.93.5% of our patients were enucleated and 6.05% exenterated. The anatomopathological results revealed 58.3% of undifferentiated tumors, in 35.97% of the cases the optic nerve was involved and in 15.8% of the cases the extensionof the tumor had reached up to the surgical resection line of the optic nerve. In 41% of the cases choroid, in 25.83% ciliary-body, in 12.2% iris, in 702% sclera and in 10.1% of the patients the orbit was occupied. The bone marrow was involved in 5.03% of the cases and the CSF in 3.6% of cases.Discussion and conclusion: In Iran, patients having unilateral retinoblastoma consult at a very late stage of the tumor, 97.1% of the stage Reese V of-Ellswortb, and in 35.97% of the cases the optic nerve and orbit were involved. 30.9% of the patients were four years old or more. The main causes of the late consultation in our country is mainly the ignorance of the parents, the social and religious believes of the parents, the actual therapeutic protocols should be modified according to the extension of the tumors.