Our study is the first report of a compound heterozygote with two mutations in the same exon (exon III) of MYOC. The occurrence of two variants (both resulting in amino acid changes) in different exons of the MYOC gene has been reported earlier [36] wherein the individual had a severe phenotype including an aggressive form of POAG and an early age of onset (14 years of age). This evidence concerns the gene MYOC and open-angle glaucoma.