A family with keratoconus combined with corneal granular dystrophy is reported. The patients are a mother and her two sons. The mother and her elder son have both keratoconus and corneal granular dystrophy; the younger son has keratoconus. Thus, the keratoconus in this family is thought to be of autosomal dominant inheritance. These familial cases suggest that the gene loci for both diseases localize in proximity or have a close relationship.