The persistent hypoglossal artery is rare vascular anomalies. We report the case of a 50-year old man with right hypoglossal artery, ipsilateral hypoplasic internal carotid artery, associated with left proximal subclavian stenosis with subclavian steal syndrome. Power-Doppler-Ultra-Sonography spectral images obtained after the patient exercised the left arm showed mid-systolic deceleration with retrograde late-systolic velocities. A Computed Tomography Angiography demonstrated a proximal stenosis of the left SA, a mild right ICA hypoplasia and an anomalous artery arising from right ICA at C2–C3 level, entering the cranium via the hypoglossal canal and joining the basilar artery. Usually the presence of PHA may be completely asymptomatic, and detected as an incidental finding by CTA or MRA, but in our case its diagnosis is extremely important because it is often the only vessel supplying blood to the basilar trunk and posterior circulation.
