Atypical hemolytic uremic syndrome (HUS) is a rare disorder in children, therefore it may lead to misdiagnosis, delay in treatment, or acute kidney injury. Patients with atypical HUS present signs and symptoms of hemolytic anemia, thrombocytopenia, and higher lactate dehydrogenase and uric acid levels. The main risk factor highlighted is consanguinity, gene mutations, and viral infections. Eculizmab, a humanized anti-C5 monoclonal antibody, has been shown to be an effective treatment modality for such patients. This article will discuss atypical hemolytic uremic syndrome, present case report of a patient suffering with atypical HUS with bad prognosis and poor recovery.