OBJECTIVE To report the outcome and complications of 10 eyes of 9 children with Sturge-Weber syndrome (SWS) who underwent two-stage insertion of a Baerveldt glaucoma implant (BGI) for glaucoma. METHODS Retrospective noncomparative case series. METHODS The authors reviewed the medical records of children under the age of 14 years with SWS who underwent two-stage BGI for glaucoma at two tertiary care referral centers. METHODS Intraoperative and postoperative complications, intraocular pressure (IOP), number of glaucoma medications, visual acuity, and further surgical intervention. RESULTS Ten eyes of nine patients were included in the study. Ages of the nine patients at time of first stage BGI ranged between 6 weeks and 13 years. With average follow-up of 35 months (range, 10-50), all eyes had adequate IOP control (< or = 21 mmHg) without the need for additional glaucoma surgery. Intraocular pressure was reduced from a mean (+/- standard deviation) of 24.8 +/- 6.2 mmHg preoperatively to 16.9 +/- 2.3 mmHg at last follow-up visit (P = 0.001). The number of medications used for control of glaucoma was reduced from a mean (+/- standard deviation) of 1.8 +/- 1.0 preoperatively to 1.1 +/- 1.4 at last follow-up visit (P = 0.2). One eye had serous choroidal effusions with overlying serous retinal detachment that resolved spontaneously after 7 days with no permanent visual loss, and one eye had low choroidal effusion that lasted 4 days. There were no intraoperative or postoperative suprachoroidal hemorrhages. At last follow-up, visual acuity had improved by one or more lines in all patients in whom vision was measurable. CONCLUSIONS Two-stage BGI surgery appears to be a safe and effective treatment for refractory glaucoma in children with SWS.