The treatment of retinoblastoma is often dependent on the individual sufferer in question. Many variables must be considered by the doctors involved. The age of the child, whether or not the cancer has spread to other areas of the body such as the brain or central nervous system and if the retinoblastoma has presented in just one or both eyes are just some of the considered questions.

A lack of treatment would result in the probable death of the child, and naturally, this is not a common option. Depending on how early the retinoblastoma is diagnosed, the goal of treatment varies. Sometimes the goal is to save life. At other times, the considerations are more cosmetic such as preserving the appearance of the eye and face or perhaps more importantly, maintaining the vision in that eye or both eyes.

The most commonly used treatment for Retinoblastoma is called Enucleation. Enucleation is a procedure whereby the eye is surgically removed in order that the cancer itself can be removed. The child is put to sleep and the eye is then removed. The operation typically takes less than an hour to complete and is not painful. The areas surrounding the eye, such as brow and eyelid, are also not negatively affected.

Children who have undergone an enucleation frequently return home the same day. A ball of plastic or rubber is placed where the original eye was, in order that there is no cavity. After the eye socket heals properly, the child can then be examined for future cosmetic alterations to improve the look of the eye, which will at first appear not dissimilar to the skin underneath the lips.

The child can typically be considered for prosthesis three weeks after surgery. The prosthesis is made of a plastic substance and is designed by a technical artist to look exactly like the child's other, real eye. Because of the restrictions on muscle control, the eye does not move quite as naturally as a real eye would. Normally, these prosthetic eyes will move up and down fairly well but there may be some problems moving side to side. However this is purely cosmetic, as the eye does not do any 'seeing' for the child. As yet, there is no safe way to transplant or replace a real eye.