Neuropsychiatric symptoms in systemic lupus erythematosus (SLE) are varied. The exact etiology and appropriate treatment are difficult to determine. We describe a 43-year-old woman who was diagnosed 18 years earlier with SLE. She developed dementia, gait disturbance, urinary incontinence and deterioration of consciousness. Her cerebrospinal fluid pressure was not elevated. Brain computerized tomography scan revealed enlarged ventricle and cortical sulci. Normal pressure hydrocephalus (NPH) was diagnosed. She was treated by establishing a ventriculo-peritoneal shunt, resulting in the return of normal consciousness. But she still had slurred speech, slow mentation and poor calculation ability. Her urinary incontinence persisted. Along with a literature review on NPH, we discuss its etiology, diagnosis and treatment.