Retinoblastoma, although a rare childhood cancer, is the most common primary malignant intraocular tumor of infancy and early childhood. In 75% of the cases, retinoblastoma is unilateral, and in 25% of the cases, retinoblastoma is bilateral. Presenting signs and symptoms of retinoblastoma, genetics, diagnostic tests, and their implications are reviewed in this paper. Treatment options for retinoblastoma (enucleation, external beam radiation, radioactive plaque, cryotherapy, photocoagulation, and chemotherapy) and their nursing care are discussed. Finally, second tumors such as osteosarcomas are discussed.