Although the mechanism of hemifacial spasm (HFS) is not yet well established, vascular compression of the facial nerve root exit zone and hyperexcitability of the facial nucleus have been suggested. We report a case of HFS in the setting of coinciding intracranial hemorrhage (ICH) of the pons and proximal ligation of the contralateral vertebral artery (VA) for the treatment of a fusiform aneurysm of the distal VA and discuss the possible etiologies of HFS in this patient. A 51-year-old male with an ICH of the pons was admitted to our hospital. Neuroimaging studies revealed an incidental fusiform aneurysm of the right VA distal to the origin of the posterior inferior cerebellar artery. Eight months after proximal ligation of the VA the patient presented with intermittent spasm of the left side of his face. Pre- and post-ligation magnetic resonance angiography revealed an enlarged diameter of the VA. The spasm completely disappeared after microvascular decompression.