Septo-optic dysplasia with lissencephaly

Septo -optic dysplasia with lissencephaly rarely occurs simultaneously. We describe such a patient presenting with absent visual fixation, mild developmental delay, ophthalmological examination revealed small pale optic disc with typical double rim and B/L optic nerve hypoplasia with CT scan finding of lissencephaly and absence of septum pellucidum. The above finding leads to the diagnosis of septo-optic dysplasia with lissencephaly.