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A 21-year-old white male with Rubinstein-Taybi syndrome (RTS) underwent emergency laparotomy and volvulus reduction for a strangulated ileus. Low blood pressure, rapid heart rate and dusky skin color indicated that he was in a hypovolemic and/or septic shock state. Communication with the patient was impossible because of severe mental retardation, deafness, and blindness, and he was quite combative and agitated. Because of an urgent situation and in anticipation of a great risk of regurgitation, no sedatives or anesthetics were used for induction of anesthesia. A large dose of vecuronium with a priming principle technique was the only agent used for endotracheal intubation. Vigorous fluid replacement and appropriate catecholamine therapy were required for the perioperative management and recovery from the shock state. Any cardiac episodes which have been reported in patients with RTS, such as supra-ventricular or ventricular arrhythmia, did not occur throughout the perioperative period. Lack of communication with the patient was an obstacle in the postoperative care, such as respiratory management or the estimation for the timing of extubation. In conclusion, the preparations for a possibly difficult airway and the possible occurrence of arrhythmia were thought to be prudent for the management of the patient with RTS.