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Osteomalacia during von Recklinghausen disease is a rare presentation of an uncommon condition and has a poorly understood mechanism.The treatment of oncogenic osteomalacia includes tumour removal which, however, is not always possible.Administration of calcitriol alone is not sufficient and phosphorus intake is mandatory to improve symptoms.A 79-year-old woman presented with left retro-orbital pain, headache and blurred vision. Based on negative radiological tests, life-threatening conditions like subarachnoid haemorrhage (SAH) were rul