Granular-lattice (Avellino) corneal dystrophy has rarely been reported in the literature. It consists of a combination of granular and lattice dystrophy. We describe the histopathologic examination of the corneal button of one Japanese patient who had undergone unilateral keratoplasty because of severely decreased vision caused by what had been diagnosed clinically as granular dystrophy. However, on pathologic examination, lesions characteristic of both granular dystrophy and lattice dystrophy were found. We also describe 2 Japanese patients who had a clinical appearance characteristic of both granular and lattice dystrophy. Granular-lattice corneal dystrophy was found in a wider geographic distribution than previously proposed and should not be named after the geographic area.