Defect of the bone of the skull and/or face with herniation of (abnormal) brain tissue, sometimes combined with a rare facial cleft.
Frontal encephaloceles
The skull defect can be situated at the site of the frontal bones, the orbit or the nasal bone.
Basal encephaloceles
The skull defect is situated at the anterior skull base, and usually presents with a cleft palate and a mass (the encephalocele) in between the palatal shelves, next to hypertelorism with or without a bifid nose and a median cleft lip.