Guillain Barre Syndrome (GBS), is an acute inflammatory polyneuropathy, of autoimmune etiology that produces an inflammation in the peripheral nerves and its spinal roots. GBS generally presents as flaccid, symmetric, ascendent and areflexic palsy, along with a previous respiratory or gastrointestinal infection. It has been reported atypical forms of presentation. We describe the case of a 53 years old male patient, without previous infections in the last months, who is admitted to the emergency department presenting walking difficulties due to decreased muscle strength, pain and numbness in the lower limbs, deep tendon reflexes present, followed few days later by a right peripheral facial palsy. Examination of cerebrospinal fluid (CSF) revealed albumino-cytological dissociation, leading to diagnose GBS of atypical presentation, which was treated with intravenous immunoglobulin followed by a favorable outcome.