NM_001202543.1:c.2383C>G (p.Leu795Val) in CUX1 is classified as Likely Benign based on ACMG/AMP 2015 criteria. The variant is present in gnomAD v4.1 at low frequency (AF=0.0167%, 270/1,613,476 alleles, 0 homozygotes), meeting PM2_supporting; however, the observation in 270 alleles warrants caution in this assignment.¹ Multiple in silico predictors concordantly support a benign effect: REVEL score 0.067, BayesDel score -0.544, and SpliceAI max delta 0.00 (BP4_supporting).² Two independent clinical testing laboratories (Ambry Genetics, CeGaT) have classified this variant as Likely Benign in ClinVar VCV2371264 (BP6_supporting).³ No case reports, functional studies, or variant-specific literature were identified for this variant. All ClinVar-associated PMIDs are unrelated policy/position statements about newborn screening. With 2 supporting benign criteria (BP4, BP6) versus 1 supporting pathogenic criterion (PM2), the benign evidence outweighs the pathogenic evidence, resulting in a Likely Benign classification per generic ACMG/AMP 2015 combination rules (PMID:25741868).⁴