Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease characterized by severe thrombocytopenia, microangiopathic haemolytic anaemia, neurologic abnormalities, renal insufficiency, and fever commonly associated with infections, malignancy, drugs, and autoimmune diseases. Coexistence of TTP with systemic lupus erythematosus (SLE) is extremely rare, and the emergence of the full manifestation of SLE starting with TTP is even rarer. Black people due to genetic risk factors are at increased risk of TTP. In the present article the authors describe the case of a Nigerian woman who developed classical features of TTP as the first manifestation of SLE. The patient’s condition was stabilized after treatment with intravenous pulses of methylprednisolone and subsequently on oral prednisolone and hydroxychloroquine treatment.