Retinoblastoma is the most common primary intraocular cancer of childhood.The most common presenting age is less than 3 years. Retinoblastoma is sporadic in 94% cases, and in about 6% cases it is familial. Leukocoria is the most common presenting feature of Retinoblastoma, whereas strabismus, painfull blind eye and loss of vision are some other clinical features. A complete ophthalmic evaluation including dilated fundus examination under anesthesia should be done and by the use of different imaging modalities diagnosis of retinoblastoma should be made and confirmed. Most importantly, the exentions of the disease should be taken into the consideration, so that the best management can be delivered to the patient. Different modes of managenent of retinoblastoma are Enucleation of eye, cryotherapy, laser phototcoagulation, thermotherapy, external beam radition, brachytherapy, and systemic chemotherapy. First priority of management is to preserve the life of the child next is to presser the vision and then to minimize the complications.