Retinoblastoma is the most common intraocular cancer among pediatric ocular malignancies, which include a wide range of benign and malignant neoplasms that impact children's ocular and periocular structures. Because of their rarity, histological heterogeneity, possibility for visual loss, and potential for life-threatening metastases, these tumors present considerable diagnostic and treatment problems. Particularly for retinoblastoma, where intra-arterial and intravitreal chemotherapy, in conjunction with focused therapies, have revolutionized management paradigms, developments in molecular genetics, imaging technologies, and therapeutic approaches have significantly improved results. Despite these developments, multidisciplinary care and close monitoring are still required due to long-term morbidity, the possibility of developing second primary cancers, and genetic implications. Precision medicine techniques, such as the creation of tailored treatments and immunotherapeutic tactics, have been made possible by new discoveries on the molecular and cellular pathophysiology of ocular malignancies. There is potential for early identification, risk assessment, and individualized treatment plans with the combination of genetic profiling and biomarker-driven diagnostics. Furthermore, advances in imaging methods, such new MRI protocols and optical coherence tomography, have improved diagnostic precision and less invasively monitored therapeutic response. In order to clarify tumor biology, find new therapeutic targets, and create less harmful, vision-preserving treatments, future directions highlight the necessity of cooperative research. International registries and pediatric-specific clinical trials are crucial for producing solid evidence to direct practice. Furthermore, using artificial intelligence in predictive modeling and image analysis could improve diagnosis and treatment even more. Improving patient outcomes and quality of life requires a thorough understanding of pediatric ocular malignancies, bolstered by translational research and technological innovation.