Mulvihill-Smith syndrome is a rare and complex genetic disorder which involves different system and organ. The description of a patient born to consanguineous parents and the presence of the syndrome in both males and females, suggests of autosomal recessive inheritance. This syndrome is characterized with senile face. The signs and symptoms are include short stature, microcephaly, multiple pigmented nevi, loud and raucous voice, hypertelorism, genital anomalies, visual change, diabetes, recurrent infections, immune deficit, deafness, tumor development (gastric, tongue, pancreas and melanoma), hipodonty, intellectual disability, and orofacial-dental abnormalities. Anesthesia management is important; because of difficult airway, glycemic instability and acceptance of this patients in the geriatric patient group. There are very few anesthesia experience in MSS. Therefore; we presented the anesthesia management in a 17-year-old female patient with Mulvihill-Smith syndrome.