McArdle disease (glycogen storage disorder type V) is a rare inherited condition resulting in impaired energy metabolism. Challenges in anesthetized patients with McArdle disease include hypoglycemia, rhabdomyolysis, myoglobinuria, acute renal failure, and postoperative fatigue. We review the literature and discuss a successful anesthetic that had no perioperative complications for a patient with McArdle disease undergoing robotic-assisted lung wedge resection. Preoperatively, we obtained a complete blood count, chemistry panel, and creatine kinase level. Intraoperatively, we proceeded with general endotracheal anesthesia and monitored point-of-care electrolytes, hemoglobin, and blood glucose. The patient had an uneventful postoperative recovery and was discharged home on postoperative Day 3. Patients with McArdle disease can undergo safe surgery with appropriate perioperative planning and a well-managed anesthetic. Efforts should focus on mitigating the risks of hypoglycemia, rhabdomyolysis, myoglobinuria, acute renal failure, and postoperative fatigue.