Human echinococcosis is a severe zoonotic parasitic disease which can be fatal if left untreated. Two forms of the disease exist, namely cystic echinococcosis (CE) caused by Echinococcus granulosus and alveolar echinococcosis (AE) caused by E. multilocularis have a widespread distribution. Both forms of the disease are characterized by slow-growing, space-occupying lesions which most commonly occur in the liver with clinical features similar to those of a tumor. The disease results in financial burdens due to the direct costs of treatment and indirect costs due to morbidity effects such as decreased employment. Although this review primarily targets human disease, CE also has significant animal health costs which can add to the overall burden of disease in society. Most of the burden of disease occurs in lower income countries. A number of studies have demonstrated that both the financial costs of the disease and the burden of disease in terms of DALYs are substantial in endemic areas and in a few communities, such as pastoralists in Tibet, echinococcosis can be amongst the highest contribution to total burden of disease. On a global scale the disease burden may rival that of many better known diseases such as African trypanosomiaisis and onchocerciasis. CE is also largely preventable and often highly cost effective to do so. Despite this, echinococcosis remains a neglected disease.