We report the case of a young boy with recurrent facial edema from his third year of life, diagnosed later with hereditary angio edema (HAE), who died of severe fatal laryngeal attack at the age of eleven. The father had milder HAE attacks until the age of 30, when he was diagnosed with systemic lupus erithematosus and the oldest brother has recurrent abdominal pain attacks, with long asymptomatic period. Family history revealed another three members who died suffocated at different ages, without any diagnosis.The death of the young boy happened in a small hospital two hours after admission, while few ineffective procedures were performed. We consider that HAE severity and fatality risk were underestimated by medical staff and the fatal outcome of HAE attack in the youngest child could have been possibly prevented in a better equipped medical unit. This case highlights the need to raise the profile of HAE within the medical community and to improve prophylaxis and treatment of this potential fatal disease.