A 53-year-old Japanese woman had corneal opacification due to granular dystrophy (GCD) since childhood. Clinically, unusual bilateral confluent dense granular opacities with a peripheral rim of clear cornea were observed. Her mother and daughter have a milder form of GCD, with circumscribed opacities separated by clear stroma. Light and electron microscopy of the patient's corneal button showed that the opacities involved the corneal epithelium as well as the stroma.