We report the successful treatment of a patient with hemifacial spasm due to a tortuous vertebral artery that appeared to have developed to compensate for agenesis of the ipsilateral carotid artery. The 51-year-old man presented with a 1-year history of progressive left hemifacial spasm. His medical history was otherwise unremarkable except for untreated mild hypertension. Magnetic resonance angiography and bone window computed tomography demonstrated congenital agenesis of the left carotid artery and compression of the root exit zone of the left facial nerve by a tortuous left vertebral artery (VA). Microvascular decompression was performed via a left suboccipital craniotomy, and the offending vessel was identified using endoscopy. The vertebral artery was successfully transposed using polytetrafluoroethylene (PTFE) tape and a PTFE ball (Bard PTFE felt, Tempe, Arizona). This is the first report of a patient with hemifacial spasm caused by an ectatic VA associated with agenesis of the ipsilateral carotid artery.