The common neuropsychiatric presentations of systemic lupus erythematosus (SLE) are psychosis, seizures, cerebral ischemic attacks and headache. However, symptoms involving peripheral nervous system have been rarely reported in SLE. Twelve-year-old girl visited our hospital due to sudden weakness in lower extremities and weak facial expression with drowsiness. Motor grade was 3/5 in lower extremities and cranial nerve palsy was observed. Inpast history, she had reticular erythema in both extremities and multiple oral ulcers with dry mouth and eyes. She was febrile on 5 days before. On admission, routine laboratory examination showed leukocytosis, anemia, increased C-reactive protein in blood, pleocytosis in cerebrospinal fluid and normal findings in urine. Brain magnetic resonance image with diffusion weighted imaging and magnetic resonance angiography were normal. As meningitis or brain stem encephalitis with Guillain-Barré syndrome was suspected, intravenous antibiotics, acyclovir, dexamethasone and immunoglobulin were administered. Despite these treatments, the patient gradually lost her consciousness. On the third day, malar rash was appeared and antinuclear, anti-dsDNA, anti-SS-A, anti-SS-Band anti-nucleosome antibodies were positive in her serum. On diagnosis of neuropsychiatric SLE, high dose steroid was intravenously injected, which ameliorated most neurologic symptoms within 2 days. Salivary excretion in parotid and submandibular gland was delayed on scan. Sjögren’s syndrome was proven on biopsy of minor salivary gland. We report a rare pediatric case of neuropsychiatric SLE with Sjögren’s syndrome presenting as Bickerstaff brainstem encephalitis and being successfully treated with early steroid pulse therapy.