Familial dysautonomia (FD) is an autosomal recessive inherited disorder, predominantly affecting the Ashkenazi Jewish population that is characterized by sensory and autonomic neuropathy. The protean manifestations and perturbations result in high morbidity and mortality. However, as a result of supportive measures and centralized care, survival has improved. As surgical options are increasing to symptomatically treat FD, anesthesiologists need to be familiar with this disorder. Because the Dysautonomia Center at NYU Medical Center is a referral center for FD patients, we have attained considerable anesthetic experience with FD. This article reviews clinical features of FD that could potentially affect anesthetic management and outlines our present practices.