PPT1dci is (I) the only homozygous variant occurring in all the PRA cases homozygous for the CFA15 haplotype; (II) involves a gene known to have a role in (syndromic) retinal degeneration in dog and other species; and (III) is shown to be highly expressed in retina compared to HIVEP3. Therefore, we propose the PPT1dci variant as causative for PRA in Miniature Schnauzer. Here, HIVEP3 is linked to retinal degeneration.