A young girl (16 years old) came to our attention because she needed dental surgery for multiple cysts; she was affected by Gorlin-Goltz syndrome and by a partial deficit of clotting factor V. This syndrome is characterized by many alterations, some of which can interfere with a normal conduct of anesthesia, especially at intubation (prognathism, kyphoscoliosis, incomplete segmentation of cervical-thoracic vertebrae). Moreover this patient was heterozygote for factor V (it was reduced to 48%), so that she had some clotting alteration. We present therefore this case and the treatment undertaken.