Summary: Hereditary angioedema (HAE) is an ultra-rare disease with autosomal dominant inheritance. Angioedema is characterized by a transient vascular reaction of deep skin tissues, the subcutaneous layer or the mucosa or the submucosa with local increased permeability of blood vessels, causing swelling of the tissues. Characteristic of bradykinin-dependent angioedema is the slow build-up of edema (up to 24 hours), persistence of pain and swelling for about 3 days, spontaneous resolution, and absence of pruritus and hives. Measurements of serum levels of C1-INH, C1-INH activity and C4 proteins form the basis for the diagnosis of HAE-1/2. Treatment of hereditary angioedema includes on-demand treatment of edema attacks, short-term prophylaxis and long-term prophylaxis. In Poland, drugs for intravenous administration are available for the treatment of HAE on demand: human plasma-derived esterase inhibitor (pd-C1-INH), recombinant human C1-esterase inhibitor (rh-C1-INH) and subcutaneous administration of bradykinin receptor antagonist B2 (icatibant). For short-term prophylaxis, a human plasma-derived esterase inhibitor (pdC1-INH) is available, while for long-term prophylaxis, we can use a plasma-derived human esterase inhibitor (pd-C1-INH) or lanadelumab, which from 09.2021 can be administered in under the drug program.