Guillain-Barré syndrome includes acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, Miller Fisher syndrome and acute pandysautonomia. Plasma exchange was the first treatment in Guillain-Barrd syndrome proven to be superior to supportive treatment alone and intravenous immunoglobulin was subsequently shown to be equally effective and is now commonly used as first-line treatment. We describe a 78-year-old woman who presented with a two-day history of progressive generalised weakness and left facial nerve palsy, preceded by a flu-like illness lasting for one week. A five-day course of daily immunoglobulin (0.4 g/kg/day) was commenced without benefit and progressive clinical deterioration. Seven days after completion of immunoglobulin treatment, plasma exchange was started with an exchange of about three litres of plasma every day for three days and every second day on two further occasions. A gradual improvement of respiratory function and peripheral muscle strength was observed after the first plasma exchange and on the eighth day the patient was weaned off mechanical ventilation. This case suggests that patients with severe Guillain-Barrd syndrome may benefit from plasma exchange after immunoglobulin treatment in refractory cases. Plasma exchange should be considered early in Guillain-Barrć syndrome cases with axonal involvement, and in the recurrent or familial Guillain-Barré syndrome forms.