Purpose: The pathologic features of a variant of granular corneal dystrophy has been described in which the presence of lattice changes in addition to characteristic granular lesions has been documented. The authors investigated the mode of inheritance, natural history, and clinical manifestations of this dystrophy. Methods: A family with this condition was investigated, and a pedigree was established. Family members underwent ophthalmic examination, and ophthalmic history was obtained. In addition, pathologic examination of corneal tissue from affected patients was performed. Results: Similar to the four previously described cases, this family also traced its origins to Avellino, Italy. This autosomal dominant condition affected 27 of 92 family members, ranging in age from 5 to 77 years. Granular deposits were the earliest and most common manifestations. Lattice lesions were present in some patients with granular lesions. Older patients had anterior stromal haze between deposits, which impaired visual acuity. Recurrent granular deposits were noted in donor corneal tissue after penetrating keratoplasty for this condition. Pathologic examination of corneal tissue from affected patients confirmed the presence of hyaline material seen in granular dystrophy as well as fusiform deposits of amyloid, similar to those seen in lattice dystrophy type I. Conclusion: This study establishes the natural history and clinical manifestations of this condition.