Introduction: Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis. Objectives: Our aim was to examine the association of bullous pemphigoid and neurological diseases in Iranian bullous pemphigoid patients. Methods: The medical records of one hundred and sixty consecutive bullous pemphigoid patients who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran, from 2006 to 2011 were examined for evidence of any neurological disease. The control group comprised of 317 age- and sex-matched subjects. Results: Neurological diseases were seen in 42 (26.4%) patients with bullous pemphigoid and in 29 (9.1%) controls (odds ratio: 3.53 (2.1–5.9), P< 0.001). Comparing cases to controls, stroke was seen in 17.5% versus 4.1%, odds ratio 4.96 (2.49–9.88); dementia in 5.6% versus 1.9%, odds ratio 3.09 (1.08–8.84); Parkinson's disease in 2.5% versus 2.2%, odds ratio 1.14 (0.33–3.94); epilepsy in 2.5% versus 0.6%, odds ratio 4.04 (0.73–22.3); and multiple sclerosis in 0 versus 0.3% odds ratio 1.00 (0.98–1.01). Limitations: The main limitations of our study were referral bias, retrospective design and a rather low sample size. Conclusions: Neurological diseases in general, and stroke and dementia in particular, were significantly associated with bullous pemphigoid in our study.