Two patients with a superficial variant of granular dystrophy of the cornea occurring primarily in nongrafted eyes developed rapid progression of clinical manifestations and visual deterioration at an early age. No other members of their family were known to be affected. A clinical diagnosis of superficial corneal dystrophy, type undetermined, was made. The histopathologic features of the corneas resembled the findings seen in Reis-Bücklers dystrophy and led to some confusion in the differential diagnosis. Electron microscopic studies in both cases unequivocally established the diagnosis of granular dystrophy.