| Sl. no | Affected area | Features |
| 1 | CNS, cranium | Mental retardation 50%, convulsion 80%, hemiplegia, hemiparesis 30%, leptomeningeal angioma, gyriform calcification (tram line), neuronal loss, cerebral cortex atrophy, cerebral ischemia, and headache | 2 | Development | Developmental delay | 3 | Eye | Glaucoma 70%, coloboma of the iris, choroidal hemangioma, buphthalmos, hemianopia, dilated blood vessels, and visual loss | 4 | Oral cavity | Oral manifestation 40%, port wine stain involving gingiva, buccal mucosa, palate, and floor of mouth and tongue, macroglossia, gingival hyperplasia, bleeding gums, gingival hemangioma, periodontitis, pulpal involvement, osteohypertrophy (rarely), and pyogenic granuloma | 5 | Skin, face | Unilateral port wine stain involving areas supplied by ophthalmic and maxillary nerves that is, check, lip, and neck. | 6 | Other extra oral involvement | Port wine stains on neck, chest, abdomen, back, trunk, and extremities |
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