Acute confusional state (delirium) appears as one of the common neuropsychiatric manifestations of systemic lupus erythematosus (SLE). It has been suggested that neuropsychiatric SLE (NP-SLE) could promote the occurrence of drug-induced extrapyramidal symptoms (EPS). Atypical antipsychotics have been thought to be useful in management of delirium because of their low incidence of adverse effects including EPS. However, no reports of the use of atypical antipsychotics in delirium due to NP-SLE have been published. Here we report a case of NP-SLE presenting an acute confusional state (delirium), which was successfully managed by risperidone.