The possibility of plastic surgery in acrocephalosyndactylia syndrome during infancy is shown. Due to her main signs and symptoms and the mode of inheritance the patient suffered from an acrocephalosyndactylia syndrome type III (Saethre-Chotzen). Craniofacial surgery with frontal craniotomy, turn of frontal bone and frontal orbital advancement were performed at the age of eight months, using bone plates developed by Luhr. The reconstruction gave an aesthetically satisfactory result, and further development was normal. The condition for a good result is the cooperation between plastic surgeons, neurosurgeons, anaesthesiologists and paediatricians in specialised centres.