A 40 years old female with history of known facial angioma, and seizures presented with shortness of breath and fever. On physical examination she was found to have a very large facial swelling/ hemangioma on the right side of her face. The patient was on antiepileptics since childhood for her known condition. She was found to have airway obstruction secondary to her facial mass and also aspiration pneumonia.

CT Scan of her face and neck showing large vascular mass involving the right side of face extending inside the mouth causing pressure and posterior displacement of the tongue. A necrotic area was also noted within the facial mass. Cataract right eye with malocclusion of right side of mandible was also noted. CT head also showed AVM involving right parietal-occipital lobe.
CT of the chest showed bilateral pleural effusion with pneumonia.

Sturge-Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma.
Sturge-Weber is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development.
The diagnosis is usually obvious on account of a congenital facial cutaneous haemangioma (also known as port wine stain or facial naevus flammeus). This feature is almost always present and usually involves the ophthalmic division (V1) of the trigeminal nerve . Only rarely (~ 5%) is intracranial involvement present without associated cutaneous naevus. In the majority of cases (72%) the naevus is unilateral and ipsilateral to the intracranial abnormality. 
The most common clinical manifestation is with childhood seizures, present in 71 - 89% of cases that are often refractory to medical therapy. These usually begin in the first few years of life and are often associated with developmental delay and hemispheric symptoms including hemiplegia / hemiparesis and / or hemianopsia.
Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases. Ophthalmological examination is also essential to identify and treat ocular involvement.

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