Four patients from families in Pennsylvania, Massachusetts, and Argentina were diagnosed clinically as having granular dystrophy. Results of pathologic examination of the corneal buttons from each patient after penetrating keratoplasty confirmed granular deposits in the anterior third of the stroma. Amyloid was demonstrated within some of these granular deposits by Congo red staining with birefringence and dichroism and by electron microscopy. In addition to the morphologically granular deposits, numerous fusiform deposits identified as amyloid by histochemistry and electron microscopy and morphologically identical to those seen in lattice corneal dystrophy were detected deep to the granular deposits. It was further shown that the histochemical pattern of staining of the granular material by a series of lectins was similar to that present in corneas with lattice dystrophy. Although a relationship between these patients cannot be definitively proven, each family traces its origins to the Italian province of Avellino.