Neuro-Behçet's disease (NBD) is a potentially fatal complication of Behçet's disease (BD) that can sometimes masquerade as a primary neoplasm, aseptic meningitis or multiple sclerosis. Headache in patients with BD may portend onset of NBD, but the majority of headache in BD is benign. Clinicians who are unaware of the specific neurological manifestations of systemic inflammatory disorders like BD may fail to consider the possibility of serious intracranial pathology. We illustrate these challenges with the case of a 50-year-old woman with a history of BD who presented with headache in the absence of initial focal neurological deficits. The diagnosis of NBD was missed on multiple occasions before the correct diagnosis was made. We describe the etiology of headache in BD, the specific neurological manifestations of BD that suggest NBD, and the utility of routine neurological exams for BD patients with chronic headache. We further discuss the appropriate use of neuroimaging for headache in BD, and we recommend consideration of NBD as a diagnosis for headache in patients suspected of having an underlying systemic disease.