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OBJECTIVE: To study the outcome of children with retinoblastoma treated with primary chemotherapy. METHODS: This was a hospital-based cohort prospective study of 91 children with retinoblastoma out of which 27 children (32 eyes) fulfilled the inclusion criteria. These children were admitted to Ophthalmology Unit, Lady Reading st st Hospital, Peshawar, Pakistan during the period of 1 January 2011 to 31 December 2013. Diagnosis was established by compatible history and ultrasonographic demonstration of posterior chamber mass with calcification. CT-Scan aided in diagnosis and also excluded extra scleral/intracranial extension. Examination under anesthesia allowed classifying the tumor according to Reese-Ellsworth classification system. Children of consenting parents were started on primary chemotherapy. Secondary treatment consisted of cryotherapy and/or argon laser photocoagulation. RESULTS: The mean age of the cohort was 2.35 years. A total of 72% had advance stage IV or V disease. Secondary treatment was given in 43% eyes, whereas, 57% needed to be enucleated. Chemoreduction salvaged 43% of eyes. The mean survival in the cohort was 67.88 weeks and the Kaplan Meier cumulative 2 year survival rate is 77%. Primary chemotherapy improved survival in the cohort (none died). The test statistic (log rank is 20.04 (df-1); 2 p<0.001). But, was not attributed to preventing globe loss (x²=2.52 (df-1), p=0.112). Preventing globe loss was attributed to the stage of the disease at 2 time of diagnosis (x² =11.476 (df-1), p<0.001). CONCLUSION: Primary chemotherapy improves survival but not globe salvage in children with retinoblastoma. Invariably, the deciding variable for vision and globe salvage is the stage of disease at diagnosis. [ABSTRACT FROM AUTHOR]