The ultrastructural changes in lattice dystrophy of the cornea are mainly characterized by stromal changes. (I) Some keratocytes show distinct signs of cellular activity with a more abundant cytoplasm and more organelles, while others show a pronounced degeneration with a cytoplasm containing numerous vesicles of various electron-density. (2) There is a fine fibrillar degeneration, amyloid in nature, with strongly interwoven fine fibrils having 80–100 Å in diameter. (3) In many areas this fine fibrillar degeneration is associated with areas of mottled appearance, probably due to cross-sectioned collagen fibrils in various stages of degeneration.