CASE REPORTAn apparently well 15-year-old male from northern part of India presented in August 2017, with a history of acute-onset bilateral painless vision loss of BE. Onset to nadir was within 3 days. There was no history of pain around the eyes or on eye movement. He also gave intermittent history of headache, which was not severe before the onset of vision loss for about 3 months. According to the ophthalmological findings outside, he had bilateral disc edema with a visual acuity of 6/36 bilaterally. He was managed as a case of optic neuritis with pulse methylprednisolone and subsequently on oral prednisolone taper. He reported no significant change in acuity with the same.He presented to our emergency department 2 months after the index event with intermittent episodes of headache, palpitation, chest pain, and persisting low visual acuity with an admission blood pressure of 240/120 mm Hg and heart rate of 160 beats per minute. There was no asymmetry in upper limb blood pressure, no renal bruit, or radio-femoral delay. Neurological examination revealed normal sensorium, visual acuity of 6/36 both eyes (BE). Fundoscopic examination revealed bilateral disc edema with hard exudates, suggestive of stage 4 Keith and Wagner hypertensive retinopathy, and fields showed centrocecal scotoma in BE [Figure 1]. He had other features of end-organ damage in the form of renal failure with subnephrotic rage proteinuria and left ventricular hypertrophy. Magnetic resonance imaging (MRI) of brain contrast showed subcortical white matter T2 hyperintensities and prior right lenticular hemorrhage with normal appearing optic nerves. Cerebrospinal fluid (CSF) analysis was normal with normal opening pressure [Figure 2].Open in a separate windowFigure 1Fundus photographs before and after surgery showing resolution of hard exudates and disc edemaOpen in a separate windowFigure 2(A) T2-weighted (T2W) fluid-attenuated inversion recovery sequence brain showing white matter changes and right lenticular hemorrhage. (B) Normal optic nerves and T2W axial sequence. (C) Contrast-enhanced CT abdomen arterial phase showing (red asterisk) suprarenal masses with heterogeneous enhancementHe required intravenous antihypertensive medications to control blood pressure, and he was shifted to neuro intensive care unit (ICU) for further management. Vasculitic and connective tissue disease workup was negative, and renal artery Doppler did not reveal any stenosis. He was evaluated for endocrine causes of hypertension and was detected to have elevated urinary metanephrine levels. Computed tomography (CT) of abdomen revealed bilateral pheochromocytoma [Figure 2]. He was subsequently managed under the department of endocrinology. His antihypertensive medications were optimized and taken up for adrenalectomy, following which his blood pressure stabilized. On follow-up after 1 year, his visual acuity has improved to 6/12 BE with residual disc pallor, and resolution of hard exudates and metanephrine levels [Figure 1]. In view of bilateral pheochromocytoma, he was evaluated for genetic causes and was detected to have 3p25-26 (c.482 G > A) VHL gene chromosomal mutation consistent with VHL disease. Further follow-up has not shown any new tumors/retinal hemangioblastomas. There was no positive family history elicited. His relatives have been advised to get screened for the mutation.