Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome characterized by white matter vasogenic edema affecting the posterior occipital and parietal lobes of the brain predominantly. A 48-year-old female patient presented to ER with complaints of breathlessness and developed sudden painless loss of vision while eliciting history. The patient had a heart rate of 104/min and accelerated hypertension (BP of 220/120 mm of Hg). MRI Brain showed subcortical white matter T2/Fluid-attenuated inversion recovery hyperintensities, suggestive of PRES. The patient regained vision completely over 5 days after nitroglycerin infusion and calcium channel blockers. Beta blocker was started in view of increased BP and anxiety. Blood pressure paradoxically increased from 170/90 mm of Hg to 200/100 mm of Hg. Urine and plasma metanephrines were elevated. Contrast-enhanced computerized tomography abdomen showed locally infiltrative, retroperitoneal mass in left para-aortic prevertebral region diagnosed as paraganglioma. The patient improved with alpha blockers and surgical removal of paraganglioma. 0.1% of hypertensive patients harbor a pheochromocytoma or paraganglioma and its presentation as PRES is very rare.