Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is a non‑hereditary, non‑familial, and congenital disorder of unknown etiology. The condition belonging to the group phakomatoses is diagnosed by the triad of skin (unilateral facial port‑wine staining (PWS) often involving the ophthalmic division of trigeminal nerve), central nervous system (intracranial leptomeningeal venous angiomas), and ocular (glaucoma and choroidal hemangioma) involvement. Bilateral PWS can be seen in 10% to 30% cases of SWS implicating both ophthalmic and maxillary trigeminal nerve distributions. Bilateral SWS is a rare entity, and little is known about the course and progression of this variant. We are presenting a rare case of bilateral SWS (type II) involving the first two divisions of trigeminal nerve with late onset glaucoma but without any clinical neurological affection.

Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is a non‑hereditary, non‑familial, and congenital disorder of unknown etiology. The condition belonging to the group phakomatoses is diagnosed by the triad of skin (unilateral facial port‑wine staining (PWS) often involving the op...