Introduction and Importance:Guillain-Barrè syndrome is a life threatening postinfectious disease causing acute paralytic neuropathy that rarely presents with asymmetrical limb weakness (1%) and unilateral facial nerve palsy (4.9%).Case Presentation:A 39-year-old male presented with pain and weakness in the right lower limb with right sided facial weakness. The cranial nerve examination revealed lower motor neuron type right facial palsy (Bell ’s palsy). On rest neurological examination, he had decreased power in the right lower limb with an absent knee and ankle reflex on presentation. Later, the weakness was symmetrical in both lower limbs.Clinical Discussion:Cerebrospinal fluid analysis showed albuminocytologic dissociation with no cells and an elevated protein of 203.2 mg/dl. The nerve conduction study was abnormal in bilateral lower limbs suggesting severe demyelinating motor neuropathy. Intravenous Immunoglobulin was started with the dose of 25 gm (0.4 mg/kg) once daily for 5 days, that is, five doses. The patient started showing signs of recovery with the initial dose of immunoglobulin.Conclusion:The course of the disease usually recovers spontaneously and completely; however, plasma exchange and immunomodulatory therapy have shown improvement in patient with rapidly deteriorating symptoms.