OBJECTIVE: The authors reviewed the pathophysiology and clinical management of endemic alveolar hydatid disease in Alaskan Eskimos, incorporating recent developments in diagnosis and treatment. SUMMARY BACKGROUND DATA: Alveolar hydatid disease is a highly lethal zoonotic infection caused by the larval stage of Echinococcus multilocularis. This cestode is restricted geographically to northern climates, where foxes and small rodents represent the natural hosts. Domestic dogs also may serve as definitive hosts, and thus, transmit the parasite to humans. Human infection is characterized by the development of a cancer-like hepatic mass, which may extend to adjacent structures or metastasize to distant sites. If the infection goes untreated, mortality reaches 80%. METHODS: The medical records of all patients with alveolar hydatid disease diagnosed or treated at the Alaska Native Medical Center between 1951 and 1993 were reviewed. Forty-two cases of active disease are presented. RESULTS: Nine patients underwent resection of hepatic lesions with intent to cure, and each had a favorable result. Average post-diagnosis survival of those patients was 22 years; six still are living and free of disease. Partial resections or drainage procedures were performed in ten patients. Chemotherapy was used to augment the surgical treatment of eight patients, and four received chemotherapy alone, resulting in improved outcomes compared with historic controls. Late complications included hepatic abscess, biliary obstruction, and portal venous hypertension. CONCLUSIONS: Whereas alveolar hydatid disease rarely is encountered in other areas of North America, the biologic potential for spread of the disease may be increasing because of illegal importation of infected foxes to the Eastern seaboard. Therefore, the surgical community should maintain an awareness of the diagnosis and management of this potentially devastating parasitic infection.