purpose Sturge-Weber syndrome is a phakomatosis with involvement of the eyelids, conjunctiva, choroid, and retina. Congenital glaucoma is the most common ocular complication resulting from raised episcleral venous pressure. We present an unusual case of glaucoma induced by pupillary block in an 81-year-old woman with Sturge-Weber syndrome. Methods The patient was referred for acute loss of vision OD and right-sided headache, pain, and nausea. The visual acuity was light perception OD with an intraocular pressure (IOP) of 41 mm Hg. Slit-lamp examination showed diffuse corneal edema OD associated with pupillary occlusion leading to angle closure and acute glaucoma attack. No pathologic changes were evidenced in the left eye. Results The raised IOP was partly relieved following administration of intravenous acetazolamide 250 mg, accompanied by relief of both pain and nausea. Laser iridotomy was performed OD, reducing the IOP to 22 mm Hg. The patient underwent cataract surgery with pupil membrane removal, leading to IOP of 17 mm Hg, but visual acuity remains unchanged due to optic nerve atrophy. Conclusions This case supports the importance of involving an ophthalmologist in the management of Sturge-Weber syndrome, with monitoring of IOP, since early management of glaucoma can modify the visual outcome.