Familial dysautonomia is an inherited disorder characterized by autonomic and sensory nervous system neuropathy resulting in extremely labile blood pressure (severe hypertension followed by hypotension). As more patients with familial dysautonomia reach adulthood due to improved medical treatment, perioperative encounters of patients with familial dysautonomia will increase. This report is the first adult case to describe an anesthetic management of kidney transplantation for an adult familial dysautonomia patient. The clinical manifestations of this disease and rationale of our anesthetic management are discussed.