The c.1187_1188insCCCAGA variant in MYOC is predicted to cause a change in the length of the protein due to an in-frame insertion of 2 amino acids (p.Asp395_Glu396insAspPro). This variant is predicted to involve < 10% of the protein within the conserved olfactomedin domain, meeting PM4_Supporting. 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. There was no computational evidence predicting a damaging or benign impact of this variant on MYOC function. A previous study (PMID: 35196929) demonstrated that the Asp395_Glu396insAspPro protein had increased insolubility and 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. 3 segregations in 1 family, with juvenile open angle glaucoma (JOAG), have been reported (PMID: 23566828), which fulfilled PP1 (3-4 meioses). Only 1 proband with JOAG had been reported (PMID: 23566828), not meeting the ≥ 2 probands threshold required to meet PS4_Supporting. In summary, this variant met the criteria to receive a score of 5 and to be classified as a variant of uncertain significance (uncertain significance classification range -1 to 5) 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, PM2_Supporting, PM4_Supporting.