The pathogenesis of atypical uremic syndrome (HUS), which is rarely encountered in childhood, is poorly understood and its mortality and morbidity rates are high. A wide variety of therapeutic approaches has been attempted and the literature contains numerous conflicting reports about the results of these approaches. In a case diagnosed as recurrent atypical HUS, pulse methyl prednisolone, fresh frozen plasma infusions and plasma exchange transfusion were used at different stages of the disease with satisfactory response.