Abstract
Basal encephalocele is a rare congenital malformation. Among basal encephaloceles, the transsellar, trans-sphenoidal encephalocele is the least common subtype. We present the case of a newborn female, who presented to us with cleft lip and cleft palate. Diagnostic neuroimaging revealed the presence of transsellar trans-sphenoidal encephalocele along with agenesis of the corpus callosum. There are very few case reports of trans-sphenoidal encephalocele with corpus callosum agenesis in a patient with midline cleft lip and palate. In this report, we discuss the clinico-radiological findings of this extremely rare condition and present a brief review of the literature.