Abstract: Phacomatosis pigmentovascularis (PPV) is a congenital syndrome characterized by diffuse epidermal capillary malformation and pigmented nevus. PPV also have systemic involvement. Ocular manifestations of PPV include glaucoma, periocular erythema, retinal vascular abnormalities, choroidal hemangioma, ocular melanocytosis, iris hererochromia, iris mammillation and fundus hyperpigmentation. Extracutaneous complications of PPV are the key in the treatment. The treatment of its associated glaucoma is challenge and most still refer to the treatment of glaucoma complicated with Sturge-Weber syndrome.(Int Rev Ophthalmol, 2021, 45: 300-306)

Key words: phacomatosis pigmentovascularis, glaucoma