A 6-year-old girl with a past medical history significant for oculodentodigital dysplasia syndrome and refractive amblyopia in the left eye was referred for treatment of elevated IOP in the left eye. Visual acuity in the left eye was counting fingers, while vision in the right eye was 20/25 on presentation. Her cycloplegic refraction with retinoscopy was +1.00 + 1.00x180 OD and−7.00 + 2.00x170 OS. Examination under anesthesia (EUA) revealed elevated pressure of 11mmHG OD and 31 mmHg OS by Perkins applanation tonometry. Pressure was checked immediately upon sedating the patient so that the effects of anesthesia on intraocular pressure were minimized. Corneal diameter was 9.5 mm in both horizontal and vertical meridian in the right eye, and 11 mm horizontally by 10.5 mm vertically for the left eye. There was residual tunica vasculosa lentis noted for both eyes. Fundus examination showed a normal sized optic disc in both eyes and a cup-to-disk ratio of 0.1 OD and 0.9 OS. Ultrasound biomicroscopy (UBM) was performed with a 48 MHz probe offering 32 mm field of view, 30 degrees scanning angle, and lateral resolution of 0.05 mm (UBM Plus Guarded, Accutome Inc, Malvern, PA, USA). UBM showed ciliary body cysts only in the left eye. Gonioscopy revealed an open angle OD, but a completely sealed angle OS 360 degrees. The remainder of the intraocular exam was unremarkable. On external exam, the patient has characteristic facial features such as prominent epicanthic folds and a narrow pinched nose with hypoplastic alae nasi. In addition to the facial features, the patient also has camptodactyly with evidence of previous syndactyly. Due to the advanced stage of glaucoma on presentation and uncontrolled IOP despite timolol 0.5 % and latanoprost 0.005 % application in the left eye, decision was made to proceed with a Baerveldt Glaucoma Implant BG101-350 (Abbott Medical Optics) for her left eye. The patients’ vision and IOP in both eyes remained stable on timolol 0.5 % and latanoprost 0.005 %. Interval EUAs revealed good position of the Baerveldt glaucoma implant in the left eye. UBM of the right eye was repeated 1 year post Baerveldt Glaucoma Implant of the left eye, and revealed no ciliary body cysts. Gonioscopy of the right eye at that time showed the angle opened to ciliary body band 360°. A repeat gonioscopy of the right eye 2 years post Baerveldt Glaucoma Implant of the left eye, however, showed that the angle now opened to scleral spur temporally and inferiorly, and to only to the posterior trabecular meshwork for the remaining quadrants. During that year her IOP in the right eye began to trend to mid and upper teens. A 24-2 Humphrey automated visual field (Humphrey Visual Field, 24-2 with SITA standard, Zeiss, San Diego, CA) was performed, which despite the generalized depression and high fixation loss, did not show obvious glaucomatous changes in the right eye. Her left eye IOP and vision remained stable since her initial tube shunt placement, and her right eye IOP ranged between 13 mmHg to 19 mmHg on timolol 0.5 % and latanoprost 0.005 %. However, three-and-a-half-years post glaucoma implant in her left eye, her right eye IOP suddenly was 32 mmHg by applanation, while her vision remained unchanged at 20/25. This IOP elevation was found 5 months after a stable interval visit with acceptable IOP. Fixed- Combination timolol 0.5 %-brimoninidine 1 % was added to her regimen to replace timolol 0.5 %; However, her IOP remained elevated above 30 mmHg on the return visit one week later. An exam under anesthesia with possible Baerveldt glaucoma implant placement was recommended to the patient and parents, given this was her better seeing eye that was now having uncontrolled IOP on maximum tolerated medical therapy. Her IOP by Perkins applanation tonometry during the EUA was 25 mmHg OD and 17 mmHg OS. Her corneal thickness was measured to be 562 microns OD and 620 microns OS. Ultrasound biomicroscopy performed during the procedure revealed ciliary body cysts closing the angle in the right eye (4A–B). Gonioscopy confirmed angle closure with the majority of the angle sealed. A Baerveldt Glaucoma Implant BG101-350 was implanted in the supero-temporal quadrant at that time. The patient continues to have acceptable IOP in the low teens with retention of poor vision OS, 4 years following implantation of the tube shunt implant in her left eye. Her IOP and vision OD have been stable and acceptable in the low to mid teens for more than 6 months following the aqueous shunt implantation in her right eye with 20/25 best- corrected visual acuity, and a full and reliable automated visual field (Humphrey, 24-2 SITA algorithm). This represents a successful outcome in this complex condition.