The aim of this study was to investigate the association between Shiga toxin-producing Escherichia coli (STEC) O157 and the simultaneous presence of the virulence genes stx2 and eae in STEC from patients with gastroenteritis. In Germany, the proportion of serogroup O157 is substantially higher among STEC isolates from patients with haemolytic uraemic syndrome (HUS) than among STEC isolates from patients with gastroenteritis. The reason for this is unknown. Independent of serogroup, the virulence genes stx2 and eae have been associated with severe disease. Data collected in 2000-2001 from a Germany-wide laboratory-based surveillance system for STEC-associated gastroenteritis in patients <15 years were analysed. Overall, 18% of the STEC isolates belonged to serogroup O157. Compared with non-O157 strains, 0157 isolates were strongly associated with the simultaneous presence of both an stx2 gene and the eae gene (OR, 76; 95%CI, 27-230). Within the subset of STEC isolates that carried both virulence genes, 60% belonged to serogroup O157, a proportion similar to that found in STEC isolates from pediatric patients with HUS in Germany and Austria (67%, P=0.35). These data suggest that the more frequent carriage of both virulence genes, i.e. stx2 and eae, forms the basis of why STEC O157 predominates in patients with HUS.