The ketogenic diet is a nonpharmacologic treatment for refractory childhood epilepsy. This high fat, low protein-carbohydrate content diet produces a ketotic state. During its perioperative period, blood glucose should be monitored frequently and maintained at the lower limit in children on the ketogenic diet undergoing general anesthesia. Also, it is important to monitor serum pH or bicarbonate levels because of the risk of metabolic acidosis. For major surgery, one should consider obtaining perioperative serum chemistry, including electrolytes, calcium and albumin. We report that a pediatric dental caries patient on the ketogenic diet can safely undergo general anesthesia without increased risk of perioperative complications or increased seizure frequency.