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A 55-year-old woman presented with Guillain-Barré syndrome, nephrotic syndrome and multiple tubular dysfunction under occupational exposure to an organic solvent, which contained acetone as the principal solvent. In this case, the onset of the nephrotic syndrome and tubular dysfunction coincided with the development of the neurological manifestation. Renal biopsy demonstrated minimal change glomerulopathy with moderate tubulointerstitial nephritis. Several clearance tests that evaluated tubular transport functions revealed multiple tubular defects (including the Na+-K+-Cl– cotransporter of thick ascending limb and distal proton pump). The simultaneous occurrence of Guillain-Barré and nephrotic syndrome has previously been reported. However, both minimal glomerulopathy with multiple tubular dysfunction and progressive peripheral neuropathy under occupational exposure to an organic solvent remain unreported. Herein, we presented a rare association of minimal change nephrotic syndrome, tubular dysfunction with Guillain-Barré syndrome under the risk of exposure to an acetone-based cleansing solution; the case raises our concerns about possible chronic nephrotoxic or neurotoxic effects of common chemical organic solvents.