Uveal melanoma (UM) is one of the rare malignancies, which can be lethal despite local treatment. Treatment options available now can control UM in early stages, but once it metastasized to distant organs especially liver, prognosis is poor. Immune checkpoint inhibitors (ICIs) are the new revolutionary treatment in management of metastatic cutaneous melanoma. UM and cutaneous melanoma are similar as both of them derived from melanocytes but in terms of mutational load and expression of antigens they are distinct. In most of clinical trials of melanoma, UM patients were excluded and up till now there are only few studies regarding role of ICIs in metastatic UM. Most of these studies showed poor outcome and low survival benefit. Currently, research is going on combinational therapies and novel immunotherapy options. Here in this article we will discuss results of studies regarding ICIs in UM, ongoing trials and new immunotherapeutic options of UM.