Interestingly, all of the previously reported cases of NEDSDV involving vitreoretinopathy have been associated with truncating (nonsense, frameshift) or splicing variants in CTNNB1. Here, we report an additional seven patients with de novo previously unreported variants in CTNNB1, including two patients with vitreoretinopathy. Here, CTNNB1 is linked to vitreoretinal degeneration.