Uveal melanoma is a rare oncological disease. This incidence has remained stable for the past 50 years. There is no survival difference depending on the type of ocular treatment (enucleation versus radiotherapy versus tumour resection). Brachytherapy (Ru-106, I-125) presently remains the most common method for treating uveal melanoma. Despite adequate and early local treatment, half of the patients will develop metastatic recurrence with an average of 2.5 years after initial diagnosis. Clinical and histological prognostic factors have been identified, but some studies suggest that inactivation of BAP1 by chromosomal deletion or mutation is a key event driving metastasis development. Presently, no adjuvant treatment prevents those metastatic relapses. Nearly 90% of patients who relapse have only liver metastases. The median survival of those patients is about four months. Numerous trials evaluating the interest of exclusive liver treatment have failed to demonstrate an increase in survival, except surgery for solitary liver metastasis. Chemotherapy with dacarbazine remains the standard treatment of metastatic patients. New treatments targeting the signal transduction pathways or aiming at the stimulation of the immune system are under development.