This report describes a familial cluster of typical Haemolytic Uremic Syndrome (HUS) caused by Shiga toxin-producing E. coli (EHEC). An 18-month-old girl initially presented with haemorrhagic diarrhoea, which rapidly progressed to acute renal failure and neurological symptoms. Her elder brother subsequently developed similar symptoms. Despite early aggressive hydration and close clinical monitoring, both siblings required dialysis and transfer to a tertiary paediatric nephrology unit. The case highlights the significant virulence of EHEC strains and the clinical challenge of preventing severe systemic complications, even when medical intervention and secondary transmission precautions are promptly implemented.