CYP1B1 is a cytochrome P450 monooxygenase that catalyzes diverse metabolic pathways essential for normal physiology. Mechanistically, it uses molecular oxygen to insert one atom into substrates while reducing the second to water, with electrons provided by NADPH via cytochrome P450 reductase 1. CYP1B1 metabolizes endogenous substrates including steroid hormones (preferentially estradiol, producing 2- and 4-hydroxyestrogens) 2, testosterone and progesterone, and catalyzes all-trans retinol conversion to all-trans retinoic acid in extrahepatic tissues 1. Additionally, it epoxidizes arachidonic acid to epoxyeicosatrienoic acids functioning as lipid mediators 3, and metabolizes xenobiotics including polycyclic aromatic hydrocarbons to DNA-damaging products 4. CYP1B1 plays a critical role in retinal vascular development and trabecular meshwork homeostasis. Disease relevance is substantial: homozygous and compound heterozygous CYP1B1 mutations cause primary congenital glaucoma (PCG), while heterozygous mutations associate with primary open-angle glaucoma 5. Mutations correlate with severe disease requiring more surgical interventions and increased bilateral involvement 6. Functionally, PCG-associated mutations alter protein stability and catalytic activity, compromising steroid metabolism and potentially disrupting embryonic development 7. Cigarette smoke induces CYP1B1 expression, altering estrogen metabolism and affecting fertility 8. Recent evidence identifies CYP1B1 as a ferroptosis mediator in cholangiocarcinoma, presenting novel therapeutic opportunities 9.