The hereditary angioedema (HAE) resources communally occurrence of angioedema that could be very threat and are recurrently linked with imperative morbidity and even mortality. Sympathetic the pathophysiology of this disease is vital for appropriate diagnosis and management of these patients. The HAE is caused through mutations in the SERPING1 gene and that result in decreased plasma levels of functional C1 inhibitor. In HAE results, the huge numbers of various mutations have been elucidated. All most 15% of patients have a mutation at or near the active site of the reactive mobile loop, resulting in a protein that decreases functional action (type II HAE). In Type I HAE is caused through a diverse range of mutations, a few of which cause the nascent protein to misfold and therefore to be incapable to go in the secretory pathway. A first mediator of swelling in HAE is bradykinin, a product of the plasma contact system. The bradykinin stimulated enlarged vascular permeability via activating the bradykinin B2 receptor, which results in phosphorylation of vascular endothelial cadherin. A regulation of both the bradykinin B2 receptor and peptidases that degrade bradykinin could influence HAE disease sternness. A HAE results from mutations in the SERPING1 gene that directed to a loss of functional C1 inhibitor. The assaults of angioedema result from creation of bradykinin, which works on bradykinin B2 receptors to improve vascular permeability.