The c.10G>A variant in the glucokinase gene, GCK, causes an amino acid change of aspartic acid to asparagine at codon 4 (p.(Asp4Asn)) of NM_000162.5. GCK is defined by the ClinGen MDEP as a gene that has a low rate of benign missense variation and has pathogenic missense variants as a common mechanism of disease (PP2). This variant has a REVEL score of 0.312, which is between the ClinGen MDEP thresholds for BP4 and PP3, predicting neither a damaging nor benign impact on GCK function.The Popmax filtering allele frequency of the c.10G>A variant in gnomAD v2.1.1 is 0.00001687, which falls between ClinGen MDEP-established cutoffs for PM2_Supporting (0.000003) and BS1 (0.00004); thus, neither criterion will be applied. This variant was identified in an individual with a normal fasting glucose and HbA1c less than or equal to 5.5% (BS2; internal lab contributors). This variant has been observed in unknown phase with the variant c.835G>T p.Glu279* (PMID: 8433729), which meets the criteria for as pathogenic by the ClinGen MDEP (BP2). In summary, c.10G>A meets the criteria to be classified as likely benign for monogenic diabetes. ACMG/AMP criteria applied, as specified by the ClinGen MDEP (specification version 1.3.0, approved 8/11/2023): BS2, BP2, PP2.