We applied optical coherence tomography (OCT) in 20 index cases with SCA‐ATXN1 and 22 healthy controls (HCs), investigating qualitative changes and quantifying the peripapillary retinal nerve fiber layer (pRNFL) thickness and combined ganglion cell and inner plexiform layer (GCIP) volume as markers of optic atrophy and outer retinal layers as markers of maculopathy. This evidence concerns the gene ATXN1 and Leber hereditary optic neuropathy.