Introduction: The hereditary angioedema of type II (HAE type II) is a disease which, in the case of invasive acts such as dental extractions, can trigger potentially fatal laryngeal edema. Observation: A 64-year-old man presented to the hospital to perform dental extractions. The anamnesis revealed a history of hereditary angioedema of type II. The National Reference Center for Angioedema (CREAK) recommended a prophylactic protocol before performing this traumatic dental procedure in order to reduce the risk of post-operative edema. Discussion: The HAE type II is under- or misdiagnosed. The literature reports cases of death related to dental procedures in patients unaware of being a carrier of the disease. Conclusion: In case of certain evocative signs (recurrent edema, abdominal pain, family history, etc.), the odontologist must play an active role in the early detection of HAE type II.