We report a 85-year-old male case with persistent primitive hypoglossal artery (PPHA) who experienced vertigo and syncope attacks and had cervical internal carotid artery (ICA) stenosis at the same side. A carotid artery stenting (CAS) procedure was performed without embolic protection devices. The patient's postoperative clinical course was good. Postoperative magnetic resonance imaging showed no ischemic complications. On fifth day of surgery, the patient was discharged with no neurological symptoms. We report, to the best of our knowledge, the second case of ICA stenosis with ipsilateral carotid-basilar anastomosis treated with CAS presented in the literature. Although persistent carotid-vertebrobasilar anastomoses are very rare; awareness of the presence of this rare variation is essential for interventional radiologists, vascular and cardiovascular surgeons and neurosurgeons to avoid iatrogenic injury causing neurologic deficits, and even death. For the treatment of neurovascular pathologies, variations and anomalies which can affect treatment options or modify the procedural technique should be also considered.