Neuropsychiatric manifestations in patients with systemic lupus erythematosus are fairly common, with a prevalence of 37∼95%. Among 19 neuropsychiatric manifestations, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is quite rare, and is characterized by progressive, symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. Generally, plasma exchange and intravenous immunoglobulin are the main treatment modalities. Here, we report a case of AIDP in a 29-year-old SLE patient, who was fully recovered with a treatment of high-dose glucocorticoid and immunosuppressive agents. Ours case suggests that AIDP should be treated differently in SLE patients to avoid disastrous results.