Systemic lupus erythematosus (SLE) is an autoimmune disease with various manifestations, while its autoantibodies and immune reactions involve multiple organs. Neuropsychiatric involvement in SLE is known to be common, however, peripheral neuropathy is relatively rare. Guillain-Barre syndrome is clinically defined as an acute demyelinating peripheral neuropathy causing weakness and numbness in the legs and arms. We describe a case of Guillain-Barre syndrome with antiphospholipid syndrome and lupus nephritis. The patient was admitted with fever and diarrhea. He developed progressive weakness of the upper and lower extremities and dysarthria with characteristic nerve conduction patterns compatible with Guillain-Barre syndrome. He also had proteinuria and gangrene of the hand and toe with antiphospholipid antibody. He received intravenous immunoglobulin and plasmapheresis for progressive neuropathy, intravenous high dose steroid to control activity of SLE, and anticoagulation for antiphospholipid syndrome. Neuropsychiatric manifestation of SLE is related to lupus activity closely, so it is important to control lupus activity.