NM_001128849.1:c.1813-2A>T is a canonical splice acceptor variant at position -2 in SMARCA4, a gene where loss of function is an established germline disease mechanism causing rhabdoid tumor predisposition syndrome type 2 (RTPS2) and small cell carcinoma of the ovary hypercalcemic type (SCCOHT) [PVS1, very strong].¹ This variant is absent from gnomAD v2.1, v4.1, and gnomAD-Canada population databases (allele frequency = 0%), consistent with PM2 at moderate strength under generic ACMG/AMP 2015 criteria.² SpliceAI predicts a strong splice-disrupting effect (max delta = 1.0; DS_AL=1.0, DS_AG=0.89) and BayesDel predicts a damaging score (0.66), but PP3 is not stacked with PVS1 per ClinGen SVI guidance (PMC6185798) to avoid double-counting the same splice-effect evidence.³ This variant is absent from ClinVar and has not been reported in any publication; no external classification, functional data, segregation data, or de novo observations are available.⁴ Under generic ACMG/AMP 2015 combination rules, one very strong (PVS1) and one moderate (PM2) criterion are sufficient for a Pathogenic classification. However, transcript version discrepancy (NM_001128849.1 vs NM_001128849.3) and absence of RNA-confirmed splicing impact warrant confirmatory review.⁵