Retinoblastoma is the most common ocular malignancy in childhood. The most common presenting sign is leukocoria (white pupil), which is usually first noted by family members and described as a cat’s eye appearance. Other cases present with strabismus or squint. The diagnosis depends on a thorough dilated retinal examination. The major goal of treatment is to save the patient’s life, and secondarily to salvage the eye and vision if possible. Although enucleation is a major form of treatment, other treatment modalities exist today. The choice of treatment depends on size, location and tumor extent.