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Purpose: To report a case of Evans syndrome with a vascular occlusive event leading to severe loss of vision., Case Report: A 12-year-old boy with Evans syndrome presented with painless acute loss of vision in the left eye during a period of remission from the disease. Examination showed visual acuity of hand motion in the left eye, left relative afferent pupillary defect, pale optic nerve head, and attenuated vessels. Optical coherence tomography showed thin macula in both eyes, and fundus fluorescein angiography revealed delayed filling time of the arterial phase in the left eye, with attenuation and sclerosis of the arterioles. An electrophysiological study showed an electronegative electroretinogram. Based on these findings, we reached a concurrent diagnosis of atypical retinitis pigmentosa in both eyes along with a major superimposed vascular occlusive event in the left eye leading to severe visual loss., Conclusion: This is a case describing a rare ocular complication of Evans syndrome, leading to severe loss of vision due to vascular occlusion of unknown mechanism.