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Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. We are reporting a case of 16-year-old male with sphenoid sinus Langerhan’s cell histiocytosis which presented as a sudden and painless loss of vision. It is a rare entity in the diagnosis of blindness. Delayed diagnosis and treatment can result in serious complications. The radiological features and management options are discussed with a review of the pertinent literature.