For many years, enucleation has been the most common treatment of retinoblastoma. It generally has been done on the affected eye of children with unilateral disease and on the more severely affected eye in children with bilateral disease. In recent years, there has been a trend toward earlier diagnosis of retinoblastoma, and there have been refinements in conservative therapeutic methods, such as radiotherapy, photocoagulation, and cryotherapy. Consequently, it often is possible to salvage the affected eye and retain useful vision in many patients who would have undergone enucleation in the past. This article provides an update on the diagnosis and treatment of retinoblastoma, with emphasis on certain misconceptions related to the management of this tumor. New genetic aspects and their relationship to counseling are discussed briefly.