The term septooptic dysplasia was coined in 1956 by de Morsier, who pointed out the association of optic nerve hypoplasia and absence of the septum pellucidum. Patients with this condition may present with clinical features of hypopituitarism, decreased visual acuity and neurodevelopmental disabilities that lead to this diagnosis. The case that is presented here is unusual in that this patient was initially diagnosed as having low tension glaucoma during a routine screening examination and was treated for glaucoma for over a year before he was discovered to have septooptic dysplasia, also known as de Morsier's syndrome.