Tinnitus is a discomforting condition associated with a sound in one or both ears that occurs without an external stimulus and can be either pulsatile or continuous. Vascular and non-vascular factors involve in etiology of pulsatile tinnitus (PT) that requires a careful physical examination and evaluation with proper and sophisticated imaging techniques to identify the cause(s). In clinical experience, it is known that turning neck towards affected ear decreases PT, whereas turning neck towards unaffected side increases PT in venous hum, due to bending of internal jugular vein (IJV) over the transverse process of the atlas which leads to increased blood flow. In this report, we presented a rare PT case caused directly by jugular vein stenosis, in which clinical characteristics were in disagreement with the literature. In our case, PT markedly reduced, instead of being aggravated, when turning neck to unaffected side in a 35-year old woman. Moreover, there were axial maximal intensity above the left jugular bulb and about 85% stenosis in 3-D volume rendering images. We also discussed the differential diagnosis by multidetector computed tomography angiography with respect to its advantageous over other imagining techniques such as CT, MRI, and MRA as well as conventional angiography.