Symptoms mainly consist of decreased vision due to corneal edema.

[citation needed] Vacuoles are demonstrated in the posterior parts of the cornea.

The corneal endothelium is normally a single layer of cells that lose their mitotic potential after development is complete.

In posterior polymorphous corneal dystrophy, the endothelium is often multilayered and has several other characteristics of an epithelium, including the presence of desmosomes, tonofilaments, and microvilli.

These abnormal cells retain their ability to divide and extend onto the trabecular meshwork to cause glaucoma in up to 40% of cases.