To date, variants in three genes, zinc finger E-box binding homeobox 1 (ZEB1, OMIM ∗189909), ovo-like zinc finger 2 (OVOL2, OMIM ∗616441), and grainy head-like 2 (GRHL2, OMIM ∗608576), encoding three mutually regulated transcription factors (TFs), have been identified to cause PPCD [7–10]. Here, ZEB1 is linked to posterior polymorphous corneal dystrophy.