Hereditary angioedema due to Cl inhibitor deficiency (HAE-C1-INH) is characterized by recurrent skin swellings, abdominal pain attacks, and more rarely - potentially life-threatening laryngeal attacks. Thus, HAE-C1-INH may be associated with a significant morbidity and mortality. Over the last years, the field of research in the pathogenesis of HAE-Cl-INH has greatly expanded. This has led to new clinical trials with new therapeutic options. Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available for treating acute attacks include plasma-derived C1-INH concentrates, a bradykinin B2 receptor antagonist, and a recombinant human C1-INH. In the USA, a plasma kallikrein inhibitor has additionally been approved. Longterm prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antifibrinolytics. Plasma-derived C1-INH and a bradykinin B2-receptor antagonist are approved for self-administration at home. The number of management options for HAE-Cl-INH has increased considerably within the last few years, thus helping to alleviate the burden of disease.