Only three families with familial, isolated band keratopathy have been reported.  These were described in the mid-twentieth century and it is possible that they had underlying ocular and corneal disease.  In one family 3 of 9 children, the product of a first-cousin mating, were affected consistent with autosomal recessive inheritance.  In two of these the keratopathy was first noted during puberty while it was present at birth in the third child.

 In another family the band keratopathy was seen in a brother and sister at 11 and 16 years old.

In the third family a father and son were affected.