Background: Guillain-Barré syndrome (GBS) is a group of clinical syndromes involving acute polyneu­ropathy secondary to an immune-mediated process. The aim of the research is to study clinical profile and outcome in children aged 1-12 years presenting with GBS. Methods: It is a prospective plus retrospective observational study (5-year retrospective + 18 months prospective). All cases of GBS admitted from January 2020-july 2021 were enrolled for the study and retrospective cases were taken from case record from January 2015 onwards in a tertiary care centre. Results: Total 45 children were enrolled. The male: female ratio was 1.64:1. Mean age at presentation was 6.3 years, in a range of 1.5 -11.5 years of age. History of antecedent illness prior to the occurrence of GBS was present in 93.3% patients with upper respiratory tract infection being most common seen in 53.3% patients. Following variants of GBS were seen on NCV study performed in 34 patients, the most common was AIDP in 46.7% patients, followed by AMAN in 22.2% subjects and AMSAN in 6.7% patients. All children presented with lower limb weakness which progressed to involve upper limbs. Other associated features like bulbar cranial nerve involvement, respiratory muscle weakness, sensory symptoms and autonomic disturbance (arrythmia, hypotension, tachycardia, bradycardia) was observed in 30,12,7,16 patients respectively. Out of the total 45 patients, 12 patients required mechanical ventilation. Conclusions: Male predominance seen in GBS. AIDP the most common subtype of GBS. Respiratory distress and autonomic instability were associated with greater severity of Hughes disability score. Factors associated with poor outcome were sensory symptoms, autonomic instability, respiratory distress and bulbar cranial nerve involvement.