A case who developed Guillain-Barré syndrome (GBS) and nephrotic syndrome (NS) simultaneously is reported. In this case, the onset of nephrotic change of proteinuria coincided with the development of the neurological disturbance. Analysis of renal biopsy by light, immunofluorescent and electron microscopy showed minor glomerular abnormalities. The occurrence of GBS in association with NS is rare, and moreover most cases in the previous reports had membranous nephropathy. Only 1 case had minimal change nephrotic syndrome (MCNS). To our knowledge, this is the first report of a case beneficially treated with glucocorticoids, suggesting that immune disorder may play an important role in the association between MCNS and GBS.