The NM_001034853.2(RPGR):c.934G>C variant is a predicted missense variant encoding the substitution of aspartic acid with histidine at amino acid 312. However, the splicing impact predictor SpliceAI gives a score of 0.97 (donor loss), which is above the ClinGen X-linked IRD VCEP recommended threshold of ≥0.2 and predicts a damaging impact on splicing (PP3). The variant is located outside the +/- 1,2 dinucleotide and another variant with the same predicted splicing impact, c.934+1G>T, was previously classified as a pathogenic variant in the same donor/acceptor motif for the RPGR-related X-linked IRD VCEP (PS1_moderate). This variant is absent from hemizygous individuals in gnomAD v4.1.0 (PM2_Supporting). At least one proband harboring this variant exhibits a phenotype including childhood-onset (1), decreased central vision acuity (0.5), night blindness (0.5), optic nerve pallor (0.5), pigmentary retinopathy (0.5), rod cone dystrophy (1), X-linked inheritance (2), which together are specific for RPGR-related recessive retinopathy (6 points, PMID: 36882936, PP4). The variant has been reported to segregate with retinal dystrophy through at least 2 affected meiosis from 1 family. (PP1; PMID: 36882936).In summary, this variant is classified as likely pathogenic for RPGR-related retinopathy based on the ClinGen X-linked Inherited Retinal Disease Expert Panel Specifications to the ACMG/AMP Variant Interpretation Guidelines for RPGR Version 1.0.0: PS1_moderate, PP3, PP4, PP1, and PM2_supporting.