We studied 19 Eskimo patients with alveolar hydatid disease from the northwestern coast of Alaska for risk factors for infection with Echinococcus multilocularis. Each case-patient was matched by age and sex with 2 unrelated controls who had no clinical or serologic evidence of infection with E. multilocularis and who resided in three villages endemic for alveolar hydatid disease. Behaviors thought to increase exposure to E. multilocularis and the chronologic occurrence of these behaviors in the participant's life were assessed by a standardized questionnaire. Case-patients were more likely than controls to have owned dogs for their entire lives (odds ratio 6.00, P < 0.05), tethered their dogs near the house (odds ratio 8.50, P < 0.05), and lived in houses built directly on the tundra rather than on gravel or a permanent foundation (odds ratio 11.00, P < 0.01). Case-patients were not more likely to have owned sled dogs, trapped or skinned foxes, or engaged in other outdoor activities away from home. These findings suggest that controlling the parasite in the domestic dog population, as well as controlling the dog population itself, are important aspects of preventing alveolar hydatid disease in the northwestern Native Alaskan population.