NM_001015877.1:c.1003A>T (p.Arg335Ter) introduces a premature termination codon in PHF6, a gene in which loss of function causes Börjeson-Forssman-Lehmann syndrome (X-linked intellectual disability).¹ The nonsense variant lies in the terminal exon (exon 11/11) and removes only 31 C-terminal amino acids, consistent with predicted NMD escape; under PMC6185798, this is assigned PVS1 at Moderate strength.² The variant is absent from gnomAD v2.1, gnomAD v4.1, and gnomAD-Canada v1.0, meeting PM2 at Supporting strength.³ BayesDel in silico prediction score of 0.83 supports a deleterious effect, meeting PP3 at Supporting strength.⁴ The combination of PVS1_Moderate, PM2_Supporting, and PP3_Supporting yields 1 Moderate and 2 Supporting criteria. Under generic ACMG/AMP 2015 final combination rules (PMID:25741868), this does not meet the threshold for Likely Pathogenic (which requires ≥3 Moderate, or 1 Moderate + ≥4 Supporting, or a Strong criterion). The evidence is indeterminate and the variant is classified as a Variant of Uncertain Significance.⁵