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To review the current standard of care in the diagnosis and treatment of idiopathic intracranial hypertension (IIH). We also discuss recent advances in the understanding of the pathogenesis of IIH with emerging trends in management of this syndrome.There has been increasing literature suggesting a role of vitamin A, adipokines, sleep disorders, and venous sinus stenosis in the pathogenesis of IIH. Newer medical treatment options like topiramate and surgical treatment options like stereotactic ventriculoperitoneal shunting have been found to be attractive alternatives. There has also been an emerging interest in cerebral venous sinus stenting, though its role and utility remain debatable.This article seeks to present a systematic approach to the management of IIH. Many newer treatment modalities are being explored for IIH refractory to standard medical therapy, but their efficacy and safety must be demonstrated in large studies before they can be adopted as part of standard treatment.