Idiopathic intracranial hypertension (IIH) is a -condition associated with elevated intracranial pressure (ICP) and frequently presents with headaches, papilledema, and visual loss. Rarely, IIH has been reported in association with acromegaly. Although removal of the tumor may reverse this process, elevated ICP, especially in the setting of an otherwise empty sella, may result in a cerebrospinal fluid (CSF) leak that is exceedingly difficult to manage. We present the first case of a patient with a functional pituitary adenoma causing acromegaly associated with IIH and an otherwise empty sella and discuss our management paradigm for this rare condition.