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Takayasu’s disease is a chronic granulomatous arteriopathy that affects large vessels and their major branches. Nonspecific symptoms characterize the early phase, whereas findings of arterial occlusion and aneurysmal formation become manifest later. Ocular signs typically refer to retinal vascular involvement, as Takayasu arteritis or hypertensive retinopathy. We report a case of a 63-year-old woman suffering from Takayasu arteritis that complained of sudden onset of blurred vision in her left eye due to crystalline lens luxation in the vitreous cavity. The patient’s past medical history was unremarkable for trauma, personal or familiar collagenopathies. Prompt surgical management was performed and the patient reached 0 LogMAR seven days after surgery. Our case illustrates the concomitant occurrence, never reported before, of two rare conditions in the same patient, namely, Takayasu arteritis and spontaneous lens dislocation. Further research and future knowledge are needed to explain whether Takayasu arteritis could obliquely injure zonular or fibrillar structures and whether these features may be possibly related.