Hereditary angioedema (HAE) that is associated with C1 inhibitor deficiency is a rare disease, the prognosis of which is determined by upper respiratory tract involvement. In the absence of appropriate treatment, 25% of patients with this condition die of fatal asphyxia caused by laryngeal edema. Fortunately, better knowledge of the pathophysiology of the disease has enabled the development of targeted treatments, including icatibant, a bradykinin B2 receptor antagonist. The results of three double-blind, randomized, Phase III studies have indicated that icatibant is effective in treating any type of attack of HAE (cutaneous, abdominal, or laryngeal). At present, icatibant appears to cause minimal side-effects. It is administered subcutaneously, which facilitates self-administration by the patient. The next challenges include developing self-administration and gaining new indications for icatibant in treating bradykinin-related angioedema that is not associated with C1 inhibitor deficiency, such as HAE type III and angiotensin-converting enzyme inhibitor-induced angioedema.