Atypical haemolytic uremic syndrome (aHUS) is a rare condition among thrombotic microangiopathies (TMAs). Diagnosing aHUS is a challenge as there are no specific tests available to confirm the diagnosis and is often made as a diagnosis of exclusion. Whereas, one of the most common complications of HUS is acute kidney injury (AKI) which can further lead to end-stage renal disease (ESRD). Therefore, we report a case of a 12-year-old boy with aHUS and stage 3 AKI complicated with acute liver injury who had complete remission following dialysis and methylprednisolone pulse treatment. He was brought to the emergency room with a decrease of consciousness and complaints of peeling skin all over his body, yellow eyes, and tea-coloured urine. Previously, non-itching reddish spots appeared accompanied by a high fever. Physical examination showed icteric sclera, hepatomegaly, and generalized exfoliative dermatitis. Laboratory findings showed anaemia with a positive coomb test, mild thrombocytopenia, decreased kidney function (Ur: 154mg/dL; Cr: 6,94mg/dL; eGFR: 10,3ml/min/1.73m2), and increased liver enzymes. Urine analysis showed proteinuria and hematuria. Abdominal ultrasonography showed a bilateral acute parenchymatous renal disease with suspected intrahepatic cholangitis. The patient underwent hemodialysis and received methylprednisolone pulse therapy at a dose of 10 mg/KgBW and then continued with peroral prednisone for 1 mg/KgBW/day and mycophenolate mofetil as maintenance therapy and ursodeoxycholic acid for 10mg/KgBW 3 times daily. Kidney function returned to normal limits and the patient recovered well without further complication. Conclusion: Atypical haemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose as it is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical haemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy.