Hereditary angioedema (HAE) is a rare disease, little known to medical and dental practitioners, but with an increasing hospitalization rate over the years. HAE is due to a C1 esterase inhibitor deficiency/dysfunction that leads to an increased vascular permeability. The airways are the most affected, and life-threatening laryngeal swelling may occur. Episodes of HAE have no clear cause, but they can be triggered by anxiety, invasive procedures, and trauma. HAE is an important issue in oral and maxillofacial surgery, otorhinolaryngology, endoscopy, emergency medicine, and anesthesiology because even simple procedures may cause laryngeal edema. Recommendations on the management of HAE include long- and short-term prophylaxis, and treatment for acute attacks, but the importance of controlling anxiety is underestimated. Here, we report on the perioperative management of nine HAE patients scheduled for oral surgery, with a brief review of the literature on this topic.