Retinoblastoma is the most common primary intraocular tumor of childhood. The most common presenting sign of retinoblastoma is leukocoria; the second most common presenting sign is strabismus. The optometrist's ability to recognize the presenting signs and symptoms of retinoblastoma can lead to early diagnosis and expeditious treatment. Treatment modalities include enucleation, radiotherapy, cryotherapy, photocoagulation, and chemotherapy. Goals of treatment include patient survival and preservation of vision. Cooperation of the optometrist, the ophthalmologist, the ophthalmic nurse, the pediatric oncologist, and the radiation therapist plays an important role in the long-term survival of these children.