Neuropsychiatric systemic lupus erythematous (NPSLE) encompasses various psychiatric and neurological manifestations that develop in patients with systemic lupus erythematous (SLE), secondary to the involvement of the central nervous system (CNS). Although neuropsychiatric manifestations are commonly described in NPSLE, catatonia has been less frequently reported in patients with SLE. The roles of benzodiazepines (BZDs), immunosuppression, therapeutic plasma exchange (TPE), and electroconvulsive therapy (ECT) have all been reported in the management of catatonia. Furthermore, another research reported that catatonic symptoms associated with NPSLE were considerably improved by TPE. We, herein, report a case of catatonia in a patient with newly diagnosed NPSLE who exhibited a favorable prognosis through the early initiation of systemic immunosuppressants and TPE. Furthermore, we have reviewed the literature on the role of medication and plasmapheresis (PP), or TPE, in the treatment of catatonia that is associated with SLE.