It is noted that the increased central sympathetic nerve activity caused by neurovascular compression at the rostral ventrolateral medulla (RVLM) is closely related to the genesis of neurogenic hypertension. The authors present the case of a 49-year-old female with refractory neurogenic hypertension to be uncontrolled even with all kinds of oral antihypertensive medications. After approval by the Ethical Committee in a hospital, she had received an intravenous introduction of calcium antagonist and beta-blocker at home for three years. The subsequent examination detail showed increased sympathetic nerve activity and compression of the left vertebral artery (VA) at the left RVLM on magnetic resonance imaging, and therefore microvascular decompression (MVD) underwent through a left lateral suboccipital approach. The left VA was seen indenting the left RVLM. To ensure the complete decompression, the distal part of VA was moved away from RVLM to fix to the dura of the petrous bone with a glue. Her blood pressure became normalized afterwards without drugs and remained normotensive for 23 months after MVD. In order to decide the surgical indication for pure neurogenic hypertension due to neurovascular compression, a strict differential diagnosis is necessary.