A case report of a 19-year-old girl with the juvenile form of Pompe's disease, who underwent thoracic epidural anesthesia, is presented. Pompe's disease, glycogen storage disease type II, is an autosomal recessive disorder characterized by the lysosomal accumulation of glycogen. Patients with the juvenile form are distinguished by involvement of limb-girdle, and respiratory muscles without cardiac or nervous system manifestations. She had been managed with nasal intermittent positive pressure ventilation (NIPPV) and nightly ventilatory support. She had scoliosis with vertebral rotation and showed respiratory muscular weakness. In order to prevent postoperative respiratory complication and to achieve cardio- pulmonary stability, we chose the thoracic epidural block to perform appendectomy. Epidural injection of 0.8% lidocaine gave good sensory block without motor block and maintained cooperative state throughout the operation. Postoperatively, she remained well, but with NIPPV.