Classification of cranial encephalocele is based on the anatomic location of the skull defect. Basal sphenoidal encephaloceles are the least common form of encephalocele whose pathogenesis can be congenital, traumatic, tumoral or spontaneous. We present a case of a very rare basal encephalocele protruding through a defect in the greater wing of the sphenoid bone into the pterygoid fossa.