INTRODUCTION: Previous studies have described septooptic dysplasia (SOD) to 
describe patients who have optic nerve hypoplasia, the absence of septum 
pellucidum, and pituitary hypoplasia. Other rare ophthalmic associations have 
been described, such as low-tension glaucoma. We report the ocular findings of a 
patient with SOD who had high intraocular pressure (IOP) and glaucoma as a part 
of the syndrome.
OBJECTIVES: To report the ocular findings in a Puerto Rican patient with SOD and 
increased IOP.
PATIENTS AND METHODS: A patient with De Morsier syndrome underwent a 
comprehensive eye examination, Humphrey visual fields, and Stratus optical 
coherence tomography, and was referred for neuroradiologic examination. The 
patient had increased IOP, visual field loss, and asymmetric optic nerve 
hypoplasia. The IOP was lowered with topical hypotensive medications.
CONCLUSIONS: The patient with the De Morsier syndrome had poor visual acuity, 
high IOP, visual field, and optical coherence tomography results that were all 
compatible with glaucoma. Further studies comparing ocular findings in patients 
with several mutations leading to De Morsier syndrome are warranted. To our 
knowledge, this is the first report on a patient with glaucoma as a part of the 
syndrome.