Intra-ocular malignancy such as Uveal Melanoma (UM) arises from melanocytes from the uveal tract. It is paramount to detect early so as to initiate therapeutic plan as much of the survival will depend of the primary tumor size. Currently, no adjuvant therapy has demonstrated survival benefit and new insights in molecular biology, have improved our comprehension of the pathogenesis essentially through GNAQ and GNA11 mutations and downstream signaling pathways MAPK, PI3K/Akt and Hippo, representing a multitude of therapeutic application. New research has also unfolded new range of biomarkers that could be exploited in this setting so as to improve metastasis detection. Many studies and clinical trials are ongoing.