It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, intellectual disability, and ipsilateral leptomeningeal angioma (cerebral malformations and tumors).

Type 2 involvement includes a facial angioma (port wine stain) with a possibility of glaucoma developing.

[2] Imaging findings may include tram track calcifications on CT, pial angiomatosis, and hemicerebral atrophy.

The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nerve, just under the surface of the face.

When a child is born with a facial cutaneous vascular malformation covering a portion of the upper or the lower eyelids, imaging should be performed to screen for intracranial leptomeningeal angiomatosis.

The recommended dosage of latanoprost is one drop daily in the evening, which permits better diurnal IOP control than does morning instillation.

[citation needed] Although it is possible for the birthmark and atrophy in the cerebral cortex to be present without symptoms, most infants will develop convulsive seizures during their first year of life.

Studies do not support the widely held belief that seizure frequency early in life in patients who have SWS is a prognostic indicator.

[7][8][9] The Sturge-Weber Foundation's (The SWF) international mission is to improve the quality of life and care for people with Sturge–Weber syndrome and associated port wine birthmark conditions.

[10] It supports affected individuals and their families with education, advocacy, and research to promote effective management and awareness.

The SWF was founded by Kirk and Karen Ball, who began searching for answers after their daughter was diagnosed with Sturge–Weber syndrome at birth.

[11] The Brain Recovery Project was formed in 2011 to fund research and establish rehabilitation protocols to help children who have had hemispherectomy surgery reach their full potential.

The charity was instrumental in setting up a specialist Sturge Weber clinic at Great Ormond Street Hospital.