BACKGROUND: Septo-optic dysplasia, also referred to as de Morsier syndrome, is a 
congenital condition characterized by classic triad features: midline brain 
abnormalities, optic nerve hypoplasia and pituitary endocrine dysfunction. 
Sometimes, other various malformations appear within syndrome.
CASE PRESENTATION: An 11 and 1/2-year-old Caucasian Southeast European female 
patient with earlier established diagnoses of growth hormone deficiency, 
diabetes insipidus, seizures, mental retardation, optic nerve atrophy and right 
ptosis, was directed to us for consultative examination.The girl of short 
stature and low weight for her age had bilateral optic nerve hypoplasia, poor 
vision, nystagmus and right eye oculomotor palsy. Electroencephalogram revealed 
epileptic changes. Magnetic resonance imaging showed an empty sella syndrome, 
partial hypoplasia of corpus callosum, cavum of pellucid septum and diffuse 
polymicrogyria of the left temporal lobe. We found all elements of septo-optic 
dysplasia plus syndrome with right oculomotor nerve involvement.
CONCLUSION: By earlier findings and evaluation, we established a diagnosis of 
septo-optic dysplasia plus. The case confirms the existence of various 
malformations within the syndrome and the need for the cooperation of several 
specialists in the diagnosis and treatment of children with the syndrome.