The incidence of invasive group A streptococcal infections in Sweden was 6.1 per 100,000 population in 2012, the highest since the disease became notifiable in 2004. Furthermore, January and February 2013 marked a dramatic increase of cases notified, partly explainable by an increase of emm1/T1 isolates, a type previously shown to cause severe invasive disease more often than other types. Healthcare providers in Sweden and health authorities in neighbouring countries have been informed about this increase.