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To provide an overview of the current literature surrounding the detection and management of retinoblastoma as well as care for the long-term survivor to serve as a guide for advanced practice nurses.Comprehensive review of the literature on retinoblastoma including case studies, journal reviews, retrospective studies, and briefs.Retinoblastoma is a pediatric intraocular malignancy usually detected in the primary care setting. Typically asymptomatic, primary care providers will often identify the presence of retinoblastoma upon routine eye exam. Prompt diagnosis and commencement of treatment are paramount as delay in diagnosis leads to higher morbidity and mortality. Treatment options vary depending on the extent of disease.Although current survival rates for retinoblastoma exceed 90%, individual visual outcome and survival are dependent upon early detection and prompt referral. In addition to research and education of survivors and families, advanced practice nurses play a key role in early detection and maintaining the current survival rate.