The m.14598T>C (p.I26V) variant in MT-ND6 was reviewed by the Mitochondrial Disease Nuclear and Mitochondrial Variant Curation Expert Panel on August 14, 2023. There are no individuals with this variant reported in the medical literature to our knowledge. There are two prior reports in ClinVar for this variant, however details are not provided precluding consideration of these cases for this curation. As such, there are no reported de novo occurrences or large families to consider for evidence of variant segregation. This variant is present in population databases including in Mitomap GenBank sequences (7/61,168 (0.012%) and seen across five top level (single letter) haplogroups with European, Asian, and African ancestry), Helix database (26/195,983 sequences (0.013%), in addition to three heteroplasmic occurrences and seen across six top level haplogroups with European, Asian, and African ancestry), and gnomAD v3.1.2 (9/56,416 sequences (0.016%) in addition to three heteroplasmic occurrences and seen across seven top level haplogroups with European, Asian, and African ancestry). The computational predictor APOGEE gives a consensus rating of neutral with a score of 0.31 (Min=0, Max=1; APOGEE2 scores on 0.0499), which predicts no damaging effect on gene function (BP4). There are no cybrids, single fiber studies, or other key functional assays reported for this variant to date. In summary, this variant meets criteria to be classified as uncertain significance for primary mitochondrial disease inherited in a mitochondrial manner. We note that two expert panel members felt likely benign was the more appropriate classification (compared to five who agreed with uncertain significance). This classification was approved by the NICHD/NINDS U24 ClinGen Mitochondrial Disease Variant Curation Expert Panel on August 14, 2023. Mitochondrial DNA-specific ACMG/AMP criteria applied: BP4.