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We have identified a significant focus and unusual clustering of human cases of cystic echinococcosis (CE) and alveolar echinococcosis (AE) in the village of Nanwan, Xiji County, Ningxia Hui Autonomous Region, in one of the most highly endemic areas for both diseases in China. The village, a Chinese Hui Islamic community, is composed of 167 members of four extended families. A total of 28 people died (12 of echinococcosis) since the village was first settled in the 1950s. Despite similar life patterns, the number of AE and CE cases occurring in each family was different. Overall, the prevalences of AE and CE were 9% (20 cases) and 5.9% (13 cases), with a combined prevalence of 14.9%. In contrast to CE, a comparison of the prevalence of AE indicated significant differences between the four family clusters. Although suggestive that host genotype might play a role in susceptibility to AE, this hypothesis requires further investigation.