This study reports a patient affected by congenital frontoethmoidal encepha
locele. The cause of this malformation is unknown. A preoperative selective
diagnosis evaluation is necessary. The workup should include an accurate c
linical examination associated with radiological study (two- and three-dime
nsional computed tomography, magnetic resonance imaging, etc). The aim of t
he surgical treatment is to restore the functional brain tissue in the cran
ial cavity, perform dural repair, correct bone lack and other associated fa
cial malformations (hypertelorism, orbital dystopia, etc.). A multidiscipli
nary team approach is necessary to resolve the brain herniation and to corr
ect splanchnocranium malformations frequently associated with encephalocele
. Cranial flap with orbital osteotomies has been performed; this operation
permits correction of the hypertelorism and of the orbital dystopia associa
ted with this malformation. In bone reconstructions, miniplates have been u
sed as fixation devices. In adults we generally use titanium, but resorbabl
e devices are required in children because of growing tissues. A restoratio
n of craniofacial malformations with good aesthetic and functional results
is achieved with early surgery.