Alveolar echinococcosis (AE) is a rare but serious larval cestodiasis caused by the development of the Echinococcus multilocularis (E. multilocularis) larva in the liver. It requires medical and surgical management, which is often still very intensive and would greatly benefit from earlier diagnosis. The annual incidence in European countries reaches a maximum of 0.74 per 100,000 inhabitants (Swiss Jura). The parasite’s life cycle involves foxes (as well as dogs) as definitive hosts and voles as intermediate hosts. Humans, who serve as accidental intermediate hosts, become infected by ingesting raw vegetables contaminated with feces from infected definitive hosts or through direct contact with them. The current increase in the population of infected foxes, monitored by the ERZ (Entente contre la rage et autres zoonoses), and their establishment in peri-urban areas are driving recent epidemiological changes: the European endemic zone is expanding, and urban cases have been reported. EA is characterized by a clinical latency of 5 to 15 years and behaves like a slow-growing cancer. However, immunogenetic predisposing factors influence susceptibility or resistance to infection, thereby explaining the existence of abortive forms. Surgery has long been considered the only effective treatment for EA. More recently, long-term administration of albendazole (Eskazole®)—prescribed in a hospital setting for this indication—and the use of interventional radiology procedures have significantly improved the prognosis for inoperable forms of this parasitic disease. In advanced cases, a liver transplant (LT) may be offered. In 1997, a European network was established. It enabled the identification of 559 cases of EA diagnosed between 1982 and 2000. The French team was restructured in 2003 into a national network, FrancEchino. The objective of this network is to continue surveillance and, where appropriate, to identify trends regarding epidemiological determinants, clinical aspects, or therapeutic management. It is coordinated, right in the heart of the endemic zone, by a team from the Besançon University Hospital, with methodological support from a scientific steering committee and the participation of the InVS. Here we report the results of the data collected for new cases during the 2001–2005 period and compare them with those obtained for the previous period.