In systemic lupus erythematosus (SLE), the central nervous system (CNS) produces numerous and varying clinical disorders. A 14-year-old girl with the features of cerebral involvement in SLE is presented. The development of diabetes insipidus (DI), which responded to desmopressin, was an interesting finding in this case. Therapy consisting of high-dose methylprednisolone combined with cyclophosphamide was effective in improving the symptoms as well as the DI.