Uveal melanoma is a malignant tumour and is the most common primary intraocular malignancy in adult population. Although melanoma can be localized in the eyelids, tear sack or conjucitva, its most frequent primary location is uvea. Large portion of uveal melanomas (almost 90%) is localized in choroid, while smaller portion can be found on the iris or ciliary body. Melanoma is succesfully diagnosed with complete clinical examination on slit lamp using indirect ophthalmoscopy. Other imaging methods such as ultrasound, flourescent angiography, optic coherent tomography and magnetic resonance are often used to complement clinical examination as well as for confirmation of diagnosis. Therapy of melanoma has evolved beyond classic ophthalmological surgical treatment, and is complemented with various radiotherapeutic methods such as local plaque brachiotherapy, as well as with radiosurgical methods such as proton beam therapy and gamma knife. Current understanding of molecular pathways and genetic mutations such as GNAQ/GNA11 mutations are recognized as leading causes for developement of melanoma. BPAP1 mutation in monosomy 3 is an important prognostic factor of metastatic spread, and offers potential for developement of therapy and other prognostic mechanisms such as gene expression profiling (GEP). Although uveal melanoma has incidence of 2-10 cases per million, it all too frequently has lethal outcome due to its pathophysiological characteristics, tendency for developement of systemic disease due to high metastatic potential and non-existant effective therapy for systemic disease, and as such represents major problem of modern medicine.