Thrombotic thrombocytopenic purpura (TTP) is a rare dynamic life-threatening disease with systemic formation of platelet thrombi in the microvasculature within all the organs. Until recently the mortality in TTP has exceeded 90%. The progress of medical research in the last two decades has significantly improved our understanding of the pathogenesis of TTP and allowed a reduction in mortality. This paper describes a case of a 36-year-old female patient with systemic lupus erythematosus (SLE) who was admitted to our hospital for jaundice and speech disturbances. Laboratory tests revealed hemolytic anemia with a negative Coombs test. Abundant schistocytes were present in peripheral blood smear. During the first day of hospitalization progression of neurological signs was observed -the patient received high-dose corticosteroids (1000 mg of methylprednisolone daily for 5 consecutive days) and underwent plasma exchange therapy. We observed significant improvement of the patient's condition, as well as a complete resolution of clinical and laboratory abnormalities. In the following 24 months there have been no signs of the relapse of TTP. The article contains also a brief update of this unusual disease.