Guillain-Barré syndrome (GBS) is a type of acute inflammatory demyelinating polyneuropathy characterized by rapid progressive numbness and weakness of the limbs. As the clinical application of exogenous gangliosides increases, the side effects of this treatment have gradually emerged. Exogenous ganglioside-associated GBS (EG-GBS) is the most serious side effect of this treatment. The primary clinical manifestation of EG-GBS is axonal GBS, which is characterized by acute, serious and rapid progressive peripheral nerve involvement that results in limb flaccid paralysis. EG-GBS is more serious than other types of axonal GBS and has a long recovery time and poor prognosis. However, the pathogenesis of EG-GBS is currently unclear. Intravenous human immunoglobulin (hIg) is an evidence-based medical treatment that has replaced plasma exchange as the preferred treatment for EG-GBS. This treatment is administered at a recommended dose of 0.4 g/(kg·d) by continuous intravenous drip for 5 days. Although high-dose hormonal therapy is also a promising treatment for EG-GBS, its efficacy requires further evaluation. In summary, early detection, early diagnosis, early discontinuation of exogenous ganglioside, and timely application of hIg pulse therapy and rehabilitation therapy can improve the prognosis of EG-GBS.