The duration of surgery was 2 h and 10 min, and that of anesthesia was 3 h and 30 min. Extubation was performed after intravenous administration of sugammadex (4 mg/kg). Throughout the surgery, hemodynamic parameters were stable, and the operation was over without using vasopressor agents. To avoid perioperative accumulation of methylmalonic acid, the temperature was maintained above 36.0 ☌ with a heating device, and clindamycin was administered intravenously. The level was 120 mg/dl at the end of the surgery. Blood sugar level declined to 82 mg/dl during surgery, and 5% glucose was administered. Hypotonic fluid (2.5% glucose) was used in the perioperative period. The preoperative blood sugar level was 113 mg/dl. Glucose preparation was used to prevent hypoglycemia while maintaining a concentration of 100–200 mg/dl to ensure optimal dosing. Intraoperative arterial blood gas values were as follows: pH, 7.369 pCO 2, 32.9 mmHg pO 2, 182 mmHg BE, − 5.9 mEq/l Na, 140 mmol/l K, 3.8 mmol/l and Cl, 104 mmol/l. Anesthesia was maintained with a gas mixture of oxygen and air (FiO 2 0.4), 1.0 minimum alveolar anesthetic concentration sevoflurane (1.2–2%), and remifentanil (0.2–0.4 mcg/kg/min). After tracheal intubation, the patient underwent an ultrasound-guided bilateral rectus sheath block with 0.2% ropivacaine (30 ml). Anesthesia was induced using thiamylal (4.6 mg/kg), remifentanil (0.4 mcg/kg/min), and rocuronium (0.9 mg/kg). After the patient entered the operating room, electrocardiography, saturation of percutaneous oxygen, and noninvasive arterial pressure were monitored. General anesthesia was combined with a peripheral nerve block. Blood results showed anemia and renal failure: hemoglobin (Hb), 6.1 g/dl blood urea nitrogen (BUN), 56.0 mg/dl Cr, 4.04 mg/dl and estimated glomerular filtration rate (eGFR), 13 ml/min/L. Preoperative venous blood gas analysis did not indicate metabolic acidosis: pH, 7.432 PCO 2, 42.1 mmHg PO 2, 38.2 mmHg and base excess (BE), 2.9 mEq/l. In 2018, the patient was admitted to the emergency room due to a deterioration of general status from infection, and an operation for a continuous ambulatory peritoneal dialysis catheter placement was scheduled. Since 2009, her renal function gradually worsened, and in 2017, plasma creatinine (Cr) was found to be above 4.0 mg/dl. Complicating her cerebral palsy, she was repeatedly hospitalized due to acidosis and seizures. She was born after 38 weeks of gestation and weighed 2624 g without asphyxia, but was diagnosed as having severe MMA at 3 days of age due to deterioration of general status, and the presence of metabolic acidosis and hyperammonemia. The patient was a 19-year-old female with a height of 163.5 cm and a weight of 21.5 kg.