Corneal abnormalities are novel clinical feature in Wolfram syndrome.

•Corneal abnormalities similar to keratoconus are frequently associated with Wolfram syndrome.•WFS1 protein, known as wolframin, is expressed in normal human and mouse corneas.•Corneal abnormalities are present in WFS1 gene knockout mice corneas.•Mutation in the WFS1 gene may be responsible for corneal abnormalities.