This report presents the clinical and genetic profile of a patient exhibiting features highly suggestive of ADOA, with a heterozygous VUS, c.1310A>G (p.Gln437Arg) identified in OPA1. The patient's clinical presentation - including adolescent onset, bilateral vision loss, color vision deficit, optic disc pallor, significant RNFL thinning, and visual field defects - is consistent with the spectrum of findings previously described in ADOA cases associated with pathogenic OPA1 mutations. This evidence concerns the gene OPA1 and autosomal dominant optic atrophy.