**Abstract**

Retinoblastoma (Rb) is the most common primary intraocular malignancy in children with an incidence from 1:15,000 to 1:20,000 live births. It can present as a unilateral or bilateral involvement of the eyes. It is generally induced by biallelic mutation of the RB1 tumor suppressor gene that leads to malignant transformation of primitive retinal cells. The most common presentation is leukocoria, followed by strabismus. The initial assessment and future treatment of such tumor should be based on the laterality, the stage of the tumor, and the presenting age of the child. In general, the primary target of therapy is to preserve the child's life. However, preserving the globe and preserving vision should be achieved whenever it's possible. Retinoblastoma treatment has evolved from enucleating the affected globe to also involving external beam radiation therapy, cryotherapy, laser photocoagulation, thermotherapy, brachytherapy, and chemotherapy (intravitreal, intra-arterial, and systematic). This chapter is intended to discuss briefly the clinical presentation of Rb, as well as a comprehensive review about the evolution and current treatment modalities with a focus on cases with low-risk features.

**Keywords:** retinoblastoma, management, enucleation, external beam radiation therapy, brachytherapy, thermotherapy, laser photocoagulation, cryotherapy, chemotherapy
