It can be idiopathic or, more commonly, associated with (i) trisomy 21, (ii) neurofibromatosis type 1 (NF-1), (iii) SCD, (iv) prior radiation therapy to the head and neck, (v) arterial structural or inflammatory vasculopathy (in the setting of connective tissue disorders, fibromuscular dysplasia, and Takayasu disease) [6,10,11]. The gene discussed is NF1; the disease is Schnyder corneal dystrophy.