Aim：While the anti-oxidation and anti-inflammatory effect of dexmedetomidine has been reported, we could not find a case report in which glucose-6-dehydrogenase(G6PD) deficiency was treated with dexmedetomidine. We report a case of the superior labial frenulectomy under the intravenous sedation using fentanyl,midazoram and dexmedetomidine on a pediatric patient with G6PD deficiency. Methods：The patient was a 5-year-old boy (height 115cm,body weight 22kg) with G6PD deficiency. He had no previous medical history of hemolytic anemia. The patient was uncooperative with regard to dental treatment. We planned to perform frenectomy under intravenous sedation. To prevent hemolysis due to stress from certain drugs, metabolic conditions, and infections are the most effective management. Therefore, we decided to perform the procedure under intravenous sedation with fentanyl (25µg), midazolam (2mg) and dexmedetomidine (0.7µg/kg/hr). We also used 2% lidocaine (with 1/80,000 epinephrine) for local anesthesia (0.9ml). Dexmedetomidine has been reported to cause less respiratory depression than the other sedatives and to be effective for pediatric sedation.　 Result: During perioperative period, we did not find any signs of hemolysis, such as fatigue, headache, and dark urine after using this method. Therefore, no hemolysis therapy was necessary.　 Conclusion：Dexmedetomidine was safe and effective for this pediatric patient with G6PD deficiency. We suggest that Dexmedetomidine will be one of the safe drugs that can be used for pediatric patient with G6PD deficiency. Keywords: G6PD deficiency, Dexmedetomidine, sedation, pediatric patient