Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. 
UMs are usually initiated by a mutation in GNAQ or GNA11, unlike cutaneous 
melanomas, which usually harbour a BRAF or NRAS mutation. The annual incidence 
in Europe and the USA is ~6 per million population per year. Risk factors 
include fair skin, light-coloured eyes, congenital ocular melanocytosis, ocular 
melanocytoma and the BAP1-tumour predisposition syndrome. Ocular treatment aims 
at preserving the eye and useful vision and, if possible, preventing metastases. 
Enucleation has largely been superseded by various forms of radiotherapy, 
phototherapy and local tumour resection, often administered in combination. 
Ocular outcomes are best with small tumours not extending close to the optic 
disc and/or fovea. Almost 50% of patients develop metastatic disease, which 
usually involves the liver, and is usually fatal within 1 year. Although UM 
metastases are less responsive than cutaneous melanoma to chemotherapy or immune 
checkpoint inhibitors, encouraging results have been reported with partial 
hepatectomy for solitary metastases, with percutaneous hepatic perfusion with 
melphalan or with tebentafusp. Better insight into tumour immunology and 
metabolism may lead to new treatments.