An 18-month-old patient with hereditary sensory and autonomic neuropathy, type VII undergoing general anesthesia for Nissen fundoplication and gastrostomy tube is presented. This is the first reported case of a patient with this particular genetic mutation receiving general anesthesia. We presented the major intraoperative events during the procedure. The anesthetic considerations and implications of caring for a patient with this particular mutation and patients with other variations of hereditary sensory and autonomic neuropathy are also discussed. We show that a patient with de novo hereditary sensory and autonomic neuropathy, type VII without anhidrosis did not require intraoperative narcotics and did not experience bradycardia, asystole, or hemodynamic compromise.