In recent years, there have been a number of advances in the diagnosis and management of patients with malignant melanoma of the posterior uvea (ciliary body and choroid). This review provides a brief update on the current status of diagnostic modalities, such as fluorescein angiography, ultrasonography, computed tomography, magnetic resonance imaging, fine needle aspiration biopsy, and the radioactive phosphorus uptake test. Following that is a more detailed review of the current controversy regarding the natural course of uveal melanomas and the available therapeutic modalities. Current indications, techniques, complications, and results are provided for various forms of management, such as observation, laser photocoagulation, plaque radiotherapy, charged particle radiotherapy, local tumor resection, enucleation, and orbital exenteration.