Uveal melanoma(UM)is a rare but highly aggressive intraocular malignancy. Although it accounts for less than 5% of all melanoma cases, UM is the most common primary intraocular malignancy in adults. In recent years, advances in molecular targeted therapies and immunomodulatory strategies have provided new opportunities for the clinical management of UM. This review summarizes the pathogenesis, molecular biomarkers, and major therapeutic advances in UM, with particular emphasis on analyzing the research achievements and clinical application prospects of signaling pathway-targeted inhibitors, immune checkpoint inhibitors, and combination treatment strategies. The potential value of individualized treatment and the limitations of current therapeutic approaches are also discussed. Current evidence suggest that multidisciplinary intervention and precision molecular diagnosis and treatment may significantly improve the prognosis of patients with UM, providing references for future clinical research and the optimization of therapeutic strategies.