Retinoblastoma is the most common primary intraocular malignancy of childhood and represents 3–4% of all pediatric cancers. Without treatment, retinoblastoma is uniformly fatal. Early diagnosis and intervention can provide cure rates of greater than 97% in areas with advanced medical resources. Advanced disease is more common in developing countries as a result of delayed diagnosis, limited access to healthcare, and socioeconomic issues that correlate to a poorer prognosis. The management of retinoblastoma is individualized to each patient, and both the intraocular and extraocular stages must be considered. Multimodal treatment strategies with surgery, chemotherapy, and focal treatment modalities remain the mainstays of therapy. Enucleation remains a highly successful and cost-effective cure for advanced intraocular retinoblastoma with poor visual potential that is available in most centers worldwide. While emerging treatments continue to improve ocular salvage and preserve vision, the guiding principle among all specialists remains “save the life, save the eye, save the sight.”