Background and Objectives: Nitroglycerin (NTG), a donor of nitric oxide, is known to provoke migraine attacks in patients with migraine. However, this effect was not explored in patients with benign recurrent vertigo (BRV). To infer the mechanism of BRV, we evaluated provocative effects of NTG in patients with vestibular migraine (VM) and BRV compared with normal controls. Materials and Methods: Thirteen patients with recurrent vertigo, 8 with VM and 5 with BRV, and 5 healthy controls received intravenous infusion of 0.5 μg/kg/min NTG over 20 minutes. Headache intensity (visual analog scale) and associated symptoms were recorded at baseline and every 10 minutes for an hour. And the subjects were also asked to complete a headache diary every hour for another 12 hours. Results: In contrast to normal controls (2/5, 40%, p=0.035) and the patients with BRV (1/5, 20%, p=0.007), all patients with VM (8/8, 100%) had migraine attacks after NTG injection. However, there was no difference in the proportion of the patients with migraine attacks after NTG injection between normal controls and the patients with BRV. Conclusion: In contrast to the patients with VM, patients with BRV are not sensitive to nitric oxide. These results suggest that the pathophysiology of BRV may be different from that of VM.