RESUMO
BACKGROUND: Hereditary angioedema, a rare and potentially life-threatening condition, is the result of a defect in the C1 esterase inhibitor. Primary care physicians should be familiar with this condition to avoid complications and improve quality of care. METHODS: We present two cases of hereditary angioedema followed by a discussion based on a literature review of the recent guidelines and advances in this condition. OBJECTIVES: To highlight the clinical aspects, diagnosis and treatment of this condition and propose a practical local management based on the available medication. CONCLUSION: Hereditary angioedema management is still evolving. More efforts should be made concerning the drug therapy which is very costly and not available worldwide.