Retinoblastoma (Rb) is a widely spreading malignant condition during childhood across the globe. It is an intraocular tumor developing from immature cells of retina. Since retina is an outgrowth of neural tissue, advancing tumor poses threat of spread of malignant cells to orbit and neural tissue of brain. Tumor arises as a result of mutation in Rb1 gene located on chromosome 13 or due to amplification of somatic MYCN. Clinical presentation of tumor is characterized by ‘leukocoria’. Accurate diagnosis and wisely opting the therapeutic approach with minimum side effects and good prognosis is indeed the best way to deal with this malignancy.