Informed consent for scientific publication was obtained. A 37-year-old man (153 cm, 69 kg) with CIPA underwent an operation for posterior spinal fusion to treat thoracic spondylotic myelopathy. His sensory deficits included hyposensitivity to superficial and deep visceral pain, thermal hyposensitivity, and he have mild mental destress, unimpaired touch, and pressure sensitivity. Because of self-mutilation such as tongue or finger biting, his mouth and limbs were deformed; however, he lived independently and had a job. Autonomic imbalance was not remarkable. All members of his family did not have any symptoms of this disease. Genetic test was undergone and diagnosed with CIPA (HSAN IV). Presenting with symptoms of gait disorder and numbness of the lower limbs, he was diagnosed with thoracic spondylotic myelopathy. He had previously undergone no operations under general anesthesia. Laboratory tests were normal. In the first operation, we monitored electrocardiography, non-invasive blood pressure measurements, oxygen saturation, end-tidal CO2, bispectral index (BIS), and body temperature via rectal probe. Anesthetic induction was applied with intravenous propofol (3 μg/ml of target control infusion [TCI]), fentanyl (100 μg), and rocuronium (70 mg). After intubation and at the time of skin incision, the patient’s blood pressure and heart rate increased. We administered 50 μg of fentanyl. Anesthesia was maintained with propofol (1.8–2.5 μg/ml of TCI) and remifentanil (0.02 μg/kg/min) to keep the BIS between 40 and 60. In the middle of the operation the blood pressure and heart rate increased slightly while remaining within the normal range. The body temperature was maintained between 36.0 and 36.6 °C using a warming blanket with hot-air and regulation of operating room temperature. After extubation, the patient felt discomfort in the throat. The patient did not receive any opioids after the operation, and his postoperative course was uneventful. However, after the surgery, he experienced bladder and rectal disturbance. Spinal cord compression was presumed to have occurred, and laminectomy was planned. In the second operation, the anesthetic management course was almost the same. Anesthetic induction was applied with intravenous propofol (3 μg/ml of TCI), fentanyl (100 μg), and rocuronium (50 mg). After induction, we adjusted the propofol and remifentanil to maintain a stable circulatory status. After operation starting body temperature was decreased to 35.4 °C. Using warming blanket with hot-air, temperature was increased at 36.2 °C. After extubation, the patient reported no sore throat, wound pain, or shivering. He did not receive any opioids after the operation, and no perioperative complications were noted. After surgery, he was discharged and continued with daily life using a wheelchair and indwelling urinary catheter.