Uveal melanoma, the most common primary intraocular malignancy in adults, is potentially a lethal tumour. Since the development of local radiotherapy, tumour control is achieved in the majority of cases and most eyes are salvaged. Despite this, more than 50% of patients develop distant metastatic spread, mainly to the liver, and in the absence of efficient treatments to extend life, die soon after. A great deal of effort has been put into developing prognostic markers for metastatic spread and survival. Novel genetic prognostic tests, recently introduced, are now being clinically used in many ocular oncology centres worldwide. In addition, in most centres, patients are referred for systemic surveillance programs for early detection of liver metastasis. Novel treatment modalities to battle metastatic uveal melanoma are being developed and used in clinical trials. Their efficacy is yet to be proved. This review summarizes the recent developments and current challenges related to uveal melanoma management.