It is a neurocutaneous disorder with angiomas involving the leptomeninges and skin of the face (port wine stain), typically in the ophthalmic and maxillary distributions of the trigeminal nerve. The commonest presentation is seizure with developmental delay. There is soft tissue hypertrophy also. CT scan show calcifications in infants and even neonates; other findings include brain atrophy, ipsilateral choroid plexus enlargement, and abnormal draining veins. Treatment includes anticonvulsants for seizure control, symptomatic and prophylactic therapy for headache, glaucoma treatment to reduce the IOP, and laser therapy for port wine stain.