Methylmalonic acidemia (MMA) is an autosomal recessive metabolic disorder of organic acids that causes various symptoms attributed to the accumulation of methylmalonic acids. We report the general anesthetic management of a 7-year-old girl with MMA who underwent periodontal treatment prior to kidney transplantation. One of our anesthetic goals was to maintain appropriate metabolism perioperatively, so we minimized her fasting time as short as possible and administered specially adjusted milk with simple carbohydrates preoperatively to reduce catabolism and promote anabolism. Consultation and cooperation with the patient’s pediatrician were also essential, as was being prepared to appropriately manage perioperative emesis due to the minimized fasting time. This case demonstrates the importance of perioperative nutritional management for patients with MMA.