Uveal melanoma is the most common primary intraocular malignant tumor in adults. In almost 90% of cases, the tumor affects the choroid, while less often it affects the ciliary body and the iris. It is usually caused by a mutation in GNAQ or GNA11. Risk factors include fair skin, light-coloured eyes, inability to tan, ocular melanocytoma, dysplastic nevus syndrome, and BAP1 mutation. A successful diagnosis is based on a complete clinical examination on slit lamp using indirect ophthalmoscopy. Furthermore, imaging methods such as ultrasound and ultrasound biomicroscopy, angiography and fundus autofluorescence, optical coherence tomography, computerized tomography and magnetic resonance imaging complement and confirm the diagnosis. Biopsy is the method of choice if other methods prove unsuccessful. Therapeutic options for uveal melanoma are constantly developing, and accordingly, enucleation has mostly been replaced by various forms of radiotherapy, phototherapy and local tumor resection. These more conservative treatment methods offer good local tumor control. There are numerous treatment options for metastatic disease, which occurs in 50% of cases, but they are still insufficient in preventing a fatal outcome.