We report a 30-year-old woman who presented symptoms of oropharyngeal palsy and glove-stocking type sensory disturbance followed by acute cerebellar ataxia, the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), gastroenteric symptoms, urinary disturbance and orthostatic hypotension. She did not have any preceding infection. She was diagnosed as having Guillain-Barré syndrome with autonomic failure. Autonomic failure such as sinus tachycardia and nocturnal ventricular arrhythmia in addition to motor and sensory dysfunction was palliated by immunoadsorption. During the course of her illness, there were elevations of antiganglioside antibodies to GT1a and GQ1b in the IgG subclass, and to GD1b and GQ1b in the IgM subclass. The elevation of anti-GD1b antibody and anti-GQ1b antibody may be pathologically related to autonomic failure, cerebellar ataxia and SIADH.