A seven-year-old boy was referred to glaucoma service for mild ptosis and gradual decrease in the left eye vision. He had facial asymmetry, with left hemifacial hypertrophy and moderate ptosis and proptosis of the left eye. There was no history of any medical or surgical intervention; family history was unremarkable. Best-corrected visual acuity were 20/20 and 20/30 in right and left eyes, respectively. Full cycloplegic refraction was +1.75 diopters in the right eye and 3.5 diopters in the left eye. Relative afferent pupillary defect was +1 in the left eye. There was 2 mm ptosis in the left eye with normal levator palpebrae function. Hertel exophthalmometry measurements were 14 and 21 mm with bar reading of 107 mm. The axial length was 22.21 and 24.51 mm in the right and left eyes, respectively. Extraocular muscle movement was normal. On slit lamp examination, the cornea was clear and the anterior chamber (AC) depth was normal. The iris was cryptless and there was a band of hyperpigmentation at the margin of the pupil consistent with EU. Gonioscopy showed anterior iris insertion and angle dysgenesis. Since EU may be associated with systemic disorders, the whole body was examined carefully to detect any remarkable signs of probable associated diseases. Skin examination was normal, and there was no café-au-lait spot or any other sign of neurofibromatosis. Dental examination was also normal. His height and weight measurements were within normal limits for his age. His mental and intellectual abilities were age-appropriate. IOP was 13 and 28 mmHg without medication in the right and left eyes, respectively. After starting three glaucoma medications for the left eye, IOP decreased to 21 mmHg. Cup-to-disc ratio was 0.2 and 0.8 in the right and left eyes, respectively, with significant rim loss in the left eye. Horizontal corneal diameter was 12 and 12.5 mm, and central corneal thickness was 578 and 589 m in the right and left eye, respectively. Peripapillary nerve fiber layer optical coherence tomography (OCT) and perimetry were normal in the right eye, but in the left eye there was severe nerve fiber layer loss in all quadrants in peripapillary OCT and a double arcuate scotoma in perimetry. Axial and coronal orbital computerized tomography (CT) scan showed left proptosis and axial elongation of the left globe. Due to asymmetric corneal diameter and antimetropia, the clinical impression was that the glaucoma in this patient was of early onset and has been missed; hence, prompt intervention was crucial. The patient was planned for Mitomycin-C (MMC)-augmented trabeculectomy, which was performed without complications. At the one-month postoperative examination, IOP was 17 mmHg without medication, and the bleb was moderately vascularized and shallow. Although subtenon MMC was injected in the superior fornix, the bleb failed after six months, and IOP increased to 20 mmHg. Bleb needling and MMC injection were not successful, and we had to perform a shunt procedure to control the IOP. Six months after the shunt procedure, IOP was controlled with the timolol-dorzolamide fixed combination.