The c.967G>A variant in MYOC is a missense variant predicted to cause substitution of Glutamic acid by Lysine at amino acid 323 (p.Glu323Lys). This variant was not found in any population of gnomAD (v2.1.1), meeting the ≤ 0.0001 threshold set for PM2_Supporting in a population of at least 10,000 alleles. The REVEL score = 0.759, which met the ≥ 0.7 threshold for PP3, predicting a damaging effect on MYOC function. A previous study (PMID: 16466712) demonstrated that the Glu323Lys protein had reduced secretion levels compared to wild type myocilin protein and met the OddsPath threshold for PS3_Moderate (> 4.3), indicating that this variant did impact protein function. 10 segregations in 1 family, with juvenile open angle glaucoma, have been reported (PMID: 9772276), which fulfilled PP1_Moderate (≥ 5 meioses in ≥ 1 family, but not the ≥ 7 meioses in > 1 family for the strong criterion). 2 probands with juvenile or primary open angle glaucoma have been reported carrying this variant (PMIDs: 9772276, 12616399), which met PS4_Supporting (≥ 2 probands). In summary, this variant met the criteria to receive a score of 7 and to be classified as likely pathogenic (likely pathogenic classification range 6 to 9) for juvenile open angle glaucoma based on the ACMG/AMP criteria met, as specified by the ClinGen Glaucoma VCEP (v1, 12 Oct 2021): PS3_Moderate, PP1_Moderate, PP3, PS4_Supporting, PM2_Supporting.