Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with 
multiple organ involvement with high morbidity and mortality rate. Among the 
severe potential fatal complications are those of the central and peripheral 
nervous system which usually develop during the course of the disease and very 
rarely from the outset of the disease. We are reporting a rare case of 
Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first 
manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis 
with no neurological improvement with initial immunosuppressive therapy, 
plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with 
remarkable and complete recovery after the second 5-day course of IVIG.