A 22-year-old male who was affected by epidermolysis bullosa (EB) and xeroderma pigmentosa (with structural and pathological changes that preclude orotracheal intubation) underwent right upper extremity amputation and ipsilateral axillary lymphadenectomy. The patient was operated without intubation, thereby assuring an optimal state of acute postoperative pain control by regional anesthesia. Intravenous administration of ketamine and remifentanil plus low-dose sevoflurane resulted in anesthesia with spontaneous breathing by the patient. Moreover, the intraoperative brachial plexus nerve block before amputation followed by positioning of an epidural catheter to deliver continuous infusion of local anesthetics close to the cut nerves during surgery obtained a very good level of acute postoperative pain control.