High altitude headache (HAH) has been defined by the International Headache Society as a headache that appears within 24 hours after ascent to 2,500 m or higher [1••]. The headache can appear in isolation or as part of acute mountain sickness (AMS), which has more dramatic symptoms than the headache alone. If symptoms are ignored, more serious conditions such as high altitude cerebral edema (HACE), high altitude pulmonary edema (HAPE), or even death may ensue. While there is no definitive understanding of the underlying pathophysiologic mechanism, it is speculated that HAH occurs from the combination of hypoxemia-induced intracranial vasodilation and subsequent cerebral edema. There are a number of preventive measures that can be adopted prior to ascending, including acclimatization and various medications. A variety of pharmacological interventions are also available to clinicians to treat this extremely widespread condition.