Hereditary angioedema (HAE) is a rare genetic disease that results from deficiency or dysfunction of C1 inhibitor (C1-INH). This disease is characterized by sudden attacks of angioedema. When edema occurs in the pharynx or larynx, it can lead to serious airway compromise, including death. Physical and/or psychological stress can trigger an attack. Dental treatment, including tooth extraction, is also a recognized trigger. We report a case of a 20-year-old male with HAE who required impacted third molar extractions. C1-INH concentrate was administered 1 hour before surgery, which was completed under deep intravenous sedation. This report describes the anesthetic management of a patient with HAE and reviews treatment options and concerns.