A melanocytoma (magnocellular nevus) is a benign pigmented tumor that may arise wherever uveal melanocytes are present. The most commonly observed site is on or adjacent to the optic nerve head. In the past this tumor was frequently misdiagnosed as a malignant melanoma and many unnecessary enucleations were carried out. Its clinical features are now well recognized and, fortunately, specimens of melanocytomas are rarely received in ocular pathology laboratories today. A review of the literature concerning this tumor is presented, with special reference to the changing concepts in nomenclature, pathogenesis, natural history, and treatment that have evolved in the past one hundred years. We also define the clinical and histopathological features of this tumor.