Thrombotic thrombocytopenic purpura (TTP) is a life-threatening haematological condition which can be secondary to many disorders. Systemic lupus erythematosus (SLE) can present with thrombotic microangiopathy and the differentiation between them is quite difficult especially in people with no previous history of SLE. Thrombocytopenia and microangiopathic haemolytic anaemia (MAHA) characterized by schistocytes on the peripheral blood smear are the most consistent signs of TTP. Plasma exchange, the main treatment for TTP must be started urgently once the TTP diagnosis is established. We report a case of 20-year-old female with no previous medical problems, who was presented with severe illness and dizziness following tooth extraction and was found to have severe microangiopathic haemolytic anaemia, her platelet count was severely decreased and with subsequent diagnosis as TTP and plasma exchange and steroids were started and fulfilled the European League Against Rheumatism and the American College of Rheumatology 2019 (EULAR/ACR-2019) criteria for SLE. The patient showed satisfactory improvement. This study of case report was made for early diagnosis and immediate treatment of unusual presentation of SLE resulted in a good prognosis.�