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¹Uz. Dr. Gazi Üniversitesi, Göz Hastalıkları, Ankara - TÜRKİYE
²Asist. Dr., Gazi Üniversitesi, Göz Hastalıkları, Ankara - TÜRKİYE
³Prof. Dr., Gazi Üniversitesi, Göz Hastalıkları, Ankara - TÜRKİYE
⁴Doç. Dr., Gazi Üniversitesi, Göz Hastalıkları, Ankara - TÜRKİYE Phakomatosis pigmentovascularis (PPV) is a group of diseases that defi ned as the coexistence of vascularized and pigmented nevi. Patients with PPV often have ophthalmic abnormalities including ocular melanocytosis, goniodysgenesis, congenital glaucoma and choroidal melanoma. In this report, ophthalmic involvements of PPV and our approach to a PPV case with congenital glaucoma and ocular melanocytosis is being discussed. Keywords : phakomatosis, pigmentovascularis, congenital, glaucoma, melanocytosis