Background: Retinoblastoma is the most common intraocular malignancy of childhood occurring in children below five years of age. Its worldwide incidence is one in 10,000 live births. Mortality rate in developed world is 5%, in developing countries is 50% while it can reach 99% if not treated. The etiology of high mortality in developing world is multifactorial like late presentation due to lack of knowledge or poor health care facility, limited knowledge about the severity of the disease among health care workers, fewer centers for its treatment and lack of modern day treatment facilities in the hospitals. Aim: To describe the profile of retinoblastoma presentation and management in a tertiary care hospital in a developing country. Methods: A retrospective data analysis of all diagnosed retinoblastoma patients who received treatment at a tertiary care hospital since last 12 years. Results: Mortality and morbidity due to retinoblastoma has improved in last 12 years due to earlier presentation when we can still save life as well as globe in many patients. Destructive procedures like exenteration and enucleation can be avoided by going for conservative treatment like chemotherapy if they present early. Conclusion: Early presentation and proper diagnosis of retinoblastoma significantly improves the outcome of its management. Awareness campaigns among parents as well as primary health care personnel is crucial for early detection of disease so that life and globe saving measures can be done to improve mortality and morbidity.