Fifty-eight enterohemorrhagic Escherichia coli O26:H11 or O26:NM (nonmotile) strains and 44 atypical enteropathogenic E. coli O26:H11 or O26:NM strains isolated from patients in 11 countries during 52 years share a common pool of non-stx virulence genes, fitness loci, and genotypic and phenotypic diagnostic markers. These findings indicate close relatedness between these pathotypes and provide a basis for their clinical laboratory diagnosis.