Depending on the TGFBI mutation, the entire mutated KE, or its proteolytic fragments, accumulate in the corneal stroma with distinct ultrastructures forming amyloid (Lattice Corneal Dystrophies; LCD), rod-shaped crystalloid structures (Granular CD type 1 and Reis-Bucklers CD), a combination of amyloid and rod-shaped bodies (Granular CD type 2), or “curly fibers” (Thiel-Behnke CD) [3,4]. The gene discussed is TGFBI; the disease is lattice corneal dystrophy type I.