: The clinical syndrome idiopathic intracranial hypertension (IIH), also termed pseudotumor cerebri, consists of symptoms of headache, nausea, vomiting and visual field defects in combination with findings of papilledema. IIH is more commonly seen in overweight women where the rise in intracranial pressure is putatively a consequence of an endocrine-based disturbance of electrolytes. Less frequently, it can also occur in men and in the pediatric age group. Associated risk factors include primary and secondary aldosteronism, pregnancy, recombinant growth hormone (r-GH) therapy, oral contraceptives, obesity, vitamin A intoxication or deficiency, Addison disease, corticosteroid therapy or acute withdrawal of steroid therapy and Cushing disease. Herein, we review the association between these conditions and IIH working toward its having a unifying neuroendocrine hypothesis.