Septo-optic dysplasia, also known as de Morsier syndrome, is a rare congenital 
entity almost always characterized by hypoplasia/dysplasia of the optical nerve, 
chiasma or optic radiations and the complete or partial absence of the septum 
pellucidum. It may also be accompanied by other malformations, including 
multiple facial dysmorphism, midline defects, cleft lip and palate, 
musculoskeletal and other non-neurological eye features. Various cases have been 
reported which have presented various combinations of symptoms and stigmata of 
the syndrome. We here present a unique case of septo-optic dysplasia with 
familial repetition, a considerably early antenatal diagnosis and an 
accompanying omphalocele, a feature never before connected with the syndrome.