Shiga-toxin producing E. coli/Enterohemorrhagic E. coli (STEC/EHEC)-hemolytic uremic syndrome (HUS) is a common cause of community-acquired acute kidney injury in childhood with the clinical triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. After ingestion of food contaminated with STEC/EHEC, the bacteria colonize the intestine and penetrate through the attaching and effacing lesion. STEC/EHEC release the Shiga toxin (Stx), which circulates by binding erythrocytes, leukocytes and platelets and reach the target organ, the kidney. Endothelial and epithelial cells in the kidney express the receptor for Stx, such as globotriaosylceramide. Binding to the receptor initiates Stx entry into the cell via endocytosis. The active Stx A subunit inhibits protein synthesis by the 60S ribosomal subunit and induces cell death. Besides the ribotoxic effect, Stx enhances the production of inflammatory cytokines and activates the complement pathway. They exacerbate the disease in several ways. Damaged endothelial cells cause thrombosis, damage erythrocytes mechanically, and consume platelets. The treatment is mainly supportive but also directed toward the specific pathophysiology of the disease. Plasma exchange has long been given to remove excessive cytokines, however, its effectiveness is controversial. Eculizumab, a monoclonal antibody against complement factor C5, is being tried for STEC/EHEC-HUS to inhibit complement activation that occurs during STEC/EHEC-HUS. We look forward to an effective treatment.