Systemic lupus erythematosus (SLE) can present with thrombotic microangiopathy (TMA), and differentiating this from thrombotic thrombocytopenic purpura can be a challenging task. Though thrombotic–thrombocytopenic purpura (TTP) is a type of TMA demonstrating a severely reduced activity of VWF, cleaving protease ADAMTS 13 can differentiate it from other causes of TMA. Association of TTP-like TMA and catatonia in lupus is not described in literature. Neuropsychiatric lupus presenting as catatonia is uncommon. Here we describe a 41-year-old female who was presented with TTP-like TMA and catatonic symptoms, which improved with plasma-exchange, benzodiazepines, and immunosuppression.