Emanuel syndrome is a very rare recessive hereditary disorder characterized by multiple congenital anomalies, craniofacial dysmorphism, significant developmental delay and mental retardation. In this case report, we discussed our anesthetic management of a pediatric patient with Emanuel Syndrome undergoing isolated cleft palate repair operation. General anesthesia was maintained with total intravenous anesthetic techniques. Videolaryngoscopy was used for endotracheal intubation. The patient was extubated at the operating room following an uneventful intraoperative period, however she developed respiratory distress and was reintubated and transferred to pediatric intensive care unit. Anesthesiologists should be prepared for difficult airway management because of concomitant micro-retrognathia, as in our patient. Most of the problems regarding anesthetic management of this extremely rare syndrome can be reduced by a detailed preoperative assesment, preparation for diffucult airway and a careful hemodynamic monitoring.