Surveillance for the fox tapeworm, Echinococcus multilocularis, has been carried out in Sweden since 2000, with about 300 red foxes analysed annually. We report the first finding of E. multilocularis in Sweden, in a fox shot in December 2010 in the south-west of the country. A second infected fox shot in the same location was detected in March 2011. This paper describes the national monitoring programme and the ongoing work to estimate the prevalence and spread of the infection.