Background: Retinoblastoma is one of the most common pediatric cancer that manifests most often in the first 2 years of life. Methods: Published articles were reviewed to evaluate the current methods of treatment.Results: There has been a continuing progress in the management of retinoblastoma. Over the past few years there has been a shift away from the use of enucleation and external beam radiotherapy, and toward more effective chemoreduction followed by improved focal therapies. This is mostly due to the desire to avoid the loss of the globe and exposure to radiotherapy. Focal therapies and chemoreduction are most used for eyes with small to moderate retinoblastoma, and in advanced cases enukleasjon is often required. Conclusions: The progress in the management of retinoblastoma has led to newer treatments that are increasing the survival rates and preservation of the eyes, often with visual function.