The presence of pulmonary arterial hypertension (PAH) is a significant predictor of major perioperative cardiovascular complications in patients undergoing cardiac diagnostic or interventional procedure or non cardiac surgery under sedation and/or anesthesia. Factors that precipitate a pulmonary hypertensive crisis include hypoxia, hypercarbia, acidosis, hypothermia, pain and airway manipulations. Pain management is challenging in patients with significant PAH. We report the use of dexmedetomidine for sedation and analgesia in a 16 year old patient with significant pulmonary hypertension, pneumonia and impending cardiorespiratory failure. This resulted in avoidance of endotracheal intubation and positive pressure ventilation, with subsequent recovery to discharge home.