Diagnosis of infection by the larval stages of Echinococcus granulosus, E. multilocaris, and E. vogeli, has increased in most parts of the world because of improved diagnostic technology, active surveillance, and increasing rates of transmission. Specific immunodiagnostics and sophisticated imaging techniques have made diagnosis more sensitive and specific. Surgery, performed by an experienced team with adequate postoperative support, remains the mainstay of therapy; however, alternative treatments, including chemotherapy and percutaneous cyst drainage, are used increasingly to aid in the management of inoperable echinococcal disease and, in some cases, for primary therapy. This article incorporates data from widely disparate sources and attempts a summary of the state-of-the-art diagnosis and treatment of echinococcal disease.