Sedation during invasive procedures provides appropriate humanitarian care as well as facilitating the completion of procedure. Although generally safe and effective, adverse effects may occur especially in patients with co-morbid diseases. In many cases, given its rapid onset and offset, propofol is chosen to provide sedation during various invasive procedures. We present a nine-year-old, 45 kg child with Duchenne muscular dystrophy (DMD) who presented for esophagogastroduodenoscopy (EGD). Given the egg allergy, which was a relative contraindication to the use of propofol, and the potential risk of malignant hyperthermia due to DMD, a combination of dexmedetomidine and ketamine was used for procedural sedation. Dexmedetomidine was administered as a loading dose of 1 μg/kg along with a single bolus dose of ketamine (1 mg/kg). This was followed by a dexmedetomidine infusion at 0.5 μg/kg/hour. The patient tolerated the procedure well and was discharged to home. Previous reports regarding the use of dexmedetomidine and ketamine for procedural sedation are reviewed and the potential efficacy of this combination is discussed.