Uveal melanoma is the most common malignant neoplasm of the eyeball, developing from melanocytes of the uveal membrane of the eye, which is significantly different from melanoma of the conjunctiva, mucous membranes and skin. The management of this disease is therefore different from that of other forms of melanoma. The disease is most often confined to the eye and its local treatment includes radiation therapy and surgery. Some patients, despite successful local treatment, develop distant metastases, most often located in the liver. The guidelines presented here cover the principles of diagnosis, prognostic evaluation and treatment of both the disease confined to the eyeball and the disease at the metastatic stage. The principles of management of conjunctival melanoma are also discussed. The recommendations are based on a review of the literature and expert opinion, and are accompanied by an assessment of their strength and reliability.