Saethre-Chotzen syndrome is an autosomal acrocephalosyndactyly syndrome whose 
gene has been assigned to chromosome 7p (TWIST). A case of a 13-year-old girl 
with Saethre-Chotzen syndrome (ACS III) is described. The features of the 
syndrome include: turriplagiocephaly with a cranial circumference of 52 cm, 
facial asymmetry, low hairline, proptosis, antimongoloid slanting of palpebral 
fissures, nasal deviation with high bridge, angled ears, scoliosis and 
torticollis, clinodactyly of the fourth and fifth toes, large halluxes, and 
neurosensorial hypoacusia. For correction of the deformity, a cranioorbital 
remodeling was performed. The craniofacial approach with remodeling of the 
frontal bar and reduction of the turricephaly resulted in a satisfactory 
morphological and functional outcome, with complete three-dimensional reshaping 
and remodeling of the frontonasoorbital area.