Introduction : De Morsier syndrome is a rare genetic disorder. Common presenting features are optic nerve hypoplasia, pituitary dysfunction and midline brain abnormality. Other features may include generalized growth retardation, short stature, microglossia, megalostomia, strabismus, visual field defect, hypothyroidism, hypoprolactinemia and mental retardation. Care report : A 15 years old female presented with alternate exotropia since childhood and gradual blurred vision for the last five months. On examination unaided vision was 6/18p in each eye. Aided vision was 6/9p with -2.50 DS in right eye and 6/12 with -1.75 DS / -0.50 Dcyl x90® in left eye. Anterior segment reveals normal. Fundus showed double ring sign of optic disc in either eye. Intraocular pressure was 09 mm of Hg in right eye whereas 10 mm of Hg in left eye. She had alternate exotropia of 55prism diopter with spectacle .Ocular motility showed -1 restriction in adduction in each eyes. Stereopsis was absent. Colour vision was 11/24 in either eye by pseudo isochromatic ishihara test. Physical examination revealed megalostomia, microxanthia. She was mentally retardate and short stature. Height was 156 cm whereas weight was 119 kg which indicate that she was suffering from obesity. Besides she had menstrual abnormality, hearing defect, palpitation, cardiac problem and insomnia. 
Conclusion: Out of three diagnostic criteria, the patient has optic nerve hypoplasia and pituitary dysfunction. It represents that it may be a case of De Morsier syndrome. To understand full spectrum of presentation is important for early diagnosis, management, genetic counseling and provide better visual future