Studies suggest an association between neurological disorders and bullous pemphigoid. The goal of this systematic review was to characterize the occurrence of neurological disorders in patients with bullous pemphigoid. We performed a systematic review of the current English literature from 1984 to June 1, 2015 for documented cases of coexistent BP and neurological disorders. The literature search resulted in 53 articles meeting the inclusion criteria. Patients with bullous pemphigoid had an increased risk of stroke (OR: 4.43 [95% CI: 2.69-7.28]; p<0.001), dementia (OR: 5.48 [95% CI: 3.26-9.23]; p<0.001), Parkinson's (OR: 3.06 [95% CI: 1.97-4.77]; p< 0.001), and epilepsy/seizures (OR: 22.88 [95% CI: 2.64-198.21]; p = 0.0045). Neurological disorders preceded bullous pemphigoid in the majority of cases with a mean time interval of 6.7 years. The one-year mortality was increased in bullous pemphigoid patients who had concomitant stroke (OR: 2.87 [95% CI: 1.67-4.96]; p<0.001). Bullous pemphigoid patients have an increased association with neurological disorders which may increase mortality.