Retinoblastoma is the most common primary intraocular malignant tumor of childhood and was invariably fatal until relatively recently. Now though, retinoblastoma is curable in virtually all children presenting in the developed world. Furthermore, it is now possible to preserve the eye and retain functional vision, and advances in treatment now spare hundreds of children the need for treatment with systemic chemotherapy. These twelve chapters span the current field of retinoblastoma from laboratory discoveries to diagnostic advances to primary treatment through visual rehabilitation. Specific attention is given to retinoblastoma in the developing world and its implications for extraocular and metastatic retinoblastoma. Finally, the surgical importance of this disease is highlighted in the discussion of anesthetic considerations for examination under anesthesia, chemotherapy including intraarterial ophthalmic artery treatment and application of radiotherapy in primary, recurrent and orbital disease.