Neuropsychiatric-systemic manifestations are seen in pediatric patient with SLE and may substantially influence the quality of life. We experienced a neuropsychiatric lupus erythematosus （NP-SLE） 10-year-old girl case with variety symptoms in acute phase. She was hospitalized for the evaluation of prolonged headache, numbness in the fingers and anemia and diagnosed with Evans syndrome at other hospital. And then, she had convulsive status and was diagnosed as NP-SLE in our hospital. We administrated plasma exchange and intravenous methylprednisolone pulse. IL-6 level in the cerebrospinal fluid（ CSF） was very high, and anti NR-2 and Ribosomal P antibodies titers in the CSF and blood were positive. She was continued to receive prednisolone and mycophenolate mofetil, and neurological/cognitive impairment decreased. The pathogenesis and definite therapy are not well understood. Further research and case study regarding prognosis are required to understand the pediatric NP-SLE.
