Hereditary angioedema (HAE) is a life‐threatening disorder triggered by trauma or stress, which causes various organ systems to become edematous. Most mortality is observed secondary to laryngeal edema, caused by dental manipulation. This disorder requires special medical management before dental treatment. Some drugs also exacerbate the disorder. Current methods of treating patients with HAE are presented.SummaryThe patient discussed here was treated prophylactically on a short‐term basis, which consisted of C1NH replacement through 2 u FFP 12 hours before surgery, the morning of surgery, and the morning after surgery. Short‐term therapy was administered in conjunction with long‐term therapy, which consisted of 600 to 900 mg danazol daily. Severe edema was effectively treated with 4 to 6 mg intravenous EACA every 6 hours, and 4 mL racemic epinephrine every hour.Because dental treatment can be life‐threatening to patients with HAE, it is important for dental practitioners to thoroughly examine patients and their medical histories, to recognize signs of the disorder. Because acute airway edema is common in these patients, dental therapy is best delivered in a hospital or where emergency treatment is a