We successfully anesthetized a 19-year-old female with type I glycogen storage disease for hepatectomy. She had hypoglycemia and severe metabolic acidosis before surgery. General anesthesia was performed with epidural anesthesia. We used acetated Ringer solution mainly as intraoperative fluids instead of lactated Ringer solution and controlled administration of glucose determining blood glucose. The patient's plasma lactate levels, pyruvate and base excess during and after operation, were unchanged compared with those before operation. Administration of sodium bicarbonate was not necessary. In the case of metabolic acidosis due to accumulation of lactate as in glycogen storage diseases, it was useful to use acetated Ringer solution for fluid therapy.