We reviewed the records of 16 patients (19 eyes) with Sturge-Weber syndrome (SWS). Scleral pigmentation was present in 12 (63.2%) of these eyes; nevus of Ota in two (10.6%). Combined trabeculotomy-trabeculectomy was performed in 18 eyes. After a mean follow up of 42 months (range, 1 to 8 years), intraocular pressure was controlled (< or = 22 mm Hg) in 11 eyes (61.1%), and visual acuity was better than 6/60 in eight. Intraoperative hyphema occurred in four eyes (22.2%); vitreous loss in three (16.7%). Postoperative choroidal detachment occurred in three eyes (16.7%); vitreous hemorrhage in one (5.6%). In all of the cases of repeat surgery (three eyes), postoperative subconjunctival injections of 5-fluorouracil resulted in a diffuse filtering bleb. We conclude that combined trabeculotomy-trabeculectomy is a promising treatment for patients with SWS with glaucoma.