The c.1010A>G variant in MYOC is a missense variant predicted to cause substitution of Glutamine by Arginine at amino acid 337 (p.Gln337Arg). 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 = 0.743, which met the ≥ 0.7 threshold for PP3, predicting a damaging effect on MYOC function. There was no functional evidence predicting a damaging or benign impact of this variant on MYOC function. 6 segregations in 2 families, with juvenile or primary open angle glaucoma (JOAG or POAG), have been reported (PMIDs: 34923728, 9361308), which fulfilled PP1_Moderate (≥5 meioses). 4 probands with JOAG or POAG have been reported carrying this variant (PMIDs: 34923728, 32300215, 9361308), which met PS4_Supporting (≥ 2 probands). 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): PP1_Moderate, PP3, PS4_Supporting, PM2_Supporting