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The phenotypic expressions range from isolated retinal involvement to varying degrees of retinopathy, cerebral infarction with calcium depositions, nephropathy, and hepatopathy. We report a case of RVCL caused by a novel <jats:italic>TREX1<\/jats:italic> mutation. This patient's multisystem presentation, retinal involvement interpreted as \u201cretinal vasculitis,\u201d and improvement of neuroimaging abnormalities with dexamethasone led to the accepted diagnosis of a rheumatologic disorder resembling Beh\u00e7et disease. Clinicians should consider RVCL in any patient with retinal capillary obliterations associated with tumefactive brain lesions or nephropathy. 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