CPAMD8 is a member of the complement 3/alpha-2-macroglobulin protein family with primary roles in eye development and innate immunity 1. The gene encodes a 1885-amino acid protein containing a signal sequence, thioester motif, and unique Kazal-type serine proteinase inhibitor domain at its C-terminus 1. CPAMD8 is highly expressed in neural crest-derived tissues of the anterior eye segment, particularly in the nonpigmented epithelium of the iris and ciliary body, as well as in the optic nerve and retina 23. Functionally, CPAMD8 likely participates in ocular anterior segment formation and may regulate drainage structures critical for intraocular pressure homeostasis 3. Biallelic CPAMD8 variants cause autosomal recessive anterior segment dysgenesis and primary congenital glaucoma 45. Additionally, biallelic variants are significantly associated with childhood and juvenile open-angle glaucoma, pigmentary glaucoma, and primary angle-closure glaucoma, representing the second most common inherited cause of childhood glaucoma after CYP1B1 326. Affected individuals frequently present with iris abnormalities, cataracts, and retinal detachment, with most requiring surgical intervention to control elevated intraocular pressure 3.