Uveal melanoma is a tumor of neuroectodermal origin, developing from melanocytes of the uveal tract (choroid, ciliary body, and iris). This disease is a rare type of melanoma, which accounts for about 3% of all reported cases of melanoma. Uveal melanoma differs from skin melanoma and mucosal melanoma in both clinical course and molecular genetic properties. For uveal melanoma, the search for new targets of targeted antitumor therapy is actively underway. A promising area of treatment for metastatic uveal melanoma is immunotherapy with immunological response regulators (anti-PD-1 and anti-PD-L1 monoclonal antibodies) and antitumor vaccines. Selumetinib did not show any advantages in overall survival, however, it became the first targeted drug with proven clinical efficacy in metastatic uveal melanoma. The study of the fundamental mechanisms and the search for predictors of antitumor immune response in uveal melanoma are necessary for the development of new, more effective methods of immunotherapy for metastatic uveal melanoma.