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Purpose To discuss diagnosis and management in the case of a patient presenting with bilateral ischemic retinal vasculopathy associated with a previously undiagnosed IgA nephropathy. Case report In 2021, a 35-year-old male presented with a sudden onset asymmetric bilateral (OU) visual loss. Best-corrected visual acuity (BCVA) was 20/200 OD, and no light perception (NLP) OS with an associated relative afferent pupillary defect (RAPD). Slit-lamp examination (SLE) confirmed normal anterior segment anatomy OU. Indirect ophthalmoscopy and subsequent fluorescein angiography (FA) confirmed the presence of bilateral arterial attenuation, telangiectatic lesions, associated perivascular sheathing, and capillary leakage. Spectral domain optical coherence tomography (OCT) showed multiple areas of thinning of the inner retina. This constellation of diagnostic findings was highly suggestive of a bilateral ischemic retinal vasculopathy with an inflammatory vasculitis. Based on a high index of suspicion for a systemic etiology, nephrology was consulted, and a diagnosis of IgA nephropathy was confirmed by renal biopsy. Systemic immunomodulatory therapy was initiated. Conclusion Although it is among the most commonly occurring forms of glomerulonephritis leading to renal failure, IgA nephropathy rarely presents with a bilateral retinal vasculopathy. Schölein – Henoch purpura, the other primary disease associated with glomerular IgA deposition, may be indistinguishable from primary IgA nephropathy. A comprehensive retinal examination with multimodal functional and structural ophthalmic diagnostic testing in conjunction with renal biopsy was needed to confirm the diagnosis. It is critical to include primary renal pathology when formulating a differential diagnosis for cases of bilateral retinal vasculitis, particularly in young otherwise healthy patients.