Guillain-Barré syndrome (GBS) is defined as an acute, autoimmune polyradiculoneuropathy. It is a rare disease that occurs at a rate of 1.11 cases per 100,000 person-years. However, once infected, up to 20% of patients develop severe disability, and approximately 5% die. There have been reports of GBS in different cancers. Among them, there are 6 previous reports of GBS in small cell lung cancer. Here, we report a case of a 52-year-old man who was diagnosed with GBS in the setting of small cell lung cancer with chemotherapy.