The larval stages of Echinococcus granulosus and E. multilocularis are involved in parasitic diseases in humans: cystic echinococcosis (CE) ("hydatid disease") and alveolar echinococcosis (AE), respectively. Both diseases and parasites have tight links with allergy because of the immunological characteristics that contribute to maintain the larvae in their human host as well as their potential in inducing clinical anaphylactic reactions in some patients. Clinical observations in patients and data obtained from mass screenings in various countries have identified both forms of echinococcosis as "polar diseases," i.e., diseases where immunological background of the patients was related to the clinical presentation and course. In particular, abortive cases (i.e., spontaneous cures) have been found in many subjects in endemic areas. On the other hand, immune suppression was associated with severe disease. AE especially might be considered as an opportunistic infection. Experimental and clinical studies have shown that Th1-related immune response was associated with protection and Th2-related response was associated with parasite growth. Genetic characteristics of the host are related to both occurrence and severity of AE and are associated with the extent of IL-10 secretion, which is a major feature of chronic progressing echinococcosis. Anaphylactic reactions, including urticaria, edema, respiratory symptoms, and anaphylactic shock due to spontaneous or provoked rupture of the parasitic cyst, are well known in CE. Anaphylactic reactions in AE are far less frequent, and have been observed in rare cases at time of metastatic dissemination of the parasitic lesions. Echinococcus-specific IgE is present in most of the patients and associated with severity. Specific histamine release by circulating basophils stimulated with E. granulosus antigens is present in all patients with CE and AE. Echinococcus allergens include (1) AgB 12-kDa subunit, a protease inhibitor and a potent Th2 inducer; (2) Ag5, a serine protease; (3) EA 21, a specific cyclophilin, with a homology with other types of cyclophilins; (4) Eg EF-1 beta/delta an elongation factor, with a homology with Strongyloides stercoralis EF that shares the same IgE epitope. A clinical cross-reaction with Thiomucase, a mucopolysaccharidase used in arthritis treatment, has recently been published. However, despite the potential risk of allergic reactions, the dogma "never puncture a hydatid cyst" is no longer valid. International experience of therapeutic technique of "puncture, aspiration, injection, re-aspiration" of hydatid cysts developed at the beginning of the 1980s has proved to be successful in a variety of selected indications that have been reviewed by WHO recommendations. A better understanding of the immunological background of echinococcosis in humans has led to new therapeutic developments, such as immunomodulation using interferon alpha. Th2-driven immunological response and IL-10-related tolerance state are common characteristics of atopic allergy and echinococcosis. The example of echinococcosis stresses the ambiguous links that exist between parasitic and allergic diseases, and show the usefulness of comparing these diseases to better understand how immune deviation may lead to pathological events and to find new therapeutic and.or preventive agents.