Childhood-onset systemic lupus erythematosus (cSLE) constitutes about 10-20% of all lupus cases. Peripheral nervous system involvement accounts for <10% of cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as one of the initial manifestations of SLE has rarely been reported in the pediatric age group. Treatment recommendations for GBS in a lupus patient do not exist at present. An 8-year-old Filipjno girl presented with fulminant GBS, lupus nephritis class IV-S(A), myocarditis, and pulmonary hypertension. She received intravenous immunoglobulin (IVIg), methylprednisolone pulses, intravenous cyclophosphamide, and therapeutic plasma exchange (TPE). Favorable treatment response was noted after the 5th TPE and 2nd cycle of IVIg. Adjunct treatment included Digoxin, Aspirin, Enalapril, Propranolol, and Amlodipine. After 4 months from the last plasma exchange, she can talk, move all extremities but still had difficulty ambulating. Due to recurrent infections, Cyclophosphamide was shifted to Mycophenolate Mofetil (MMF) and partial remission was achieved on the 2nd month of MMF. High index of suspicion, timely interdisciplinary care and detailed diagnostic work up needs to be emphasized to overcome the difficulty in managing a rare case despite the lack of specific treatment recommendations.