Introduction Chronic mountain sickness (CMS) represents a variably reversible asynchronous syndrome of secondary polycythemia along with erythrocytosis, erythrocyte aggregation, hemoglobinemia, hemoconcentration, thrombocytopenia, increased whole blood viscosity, fibrinogenemia, prothrombotic state, pulmonary and systemic hypertension, and congestive heart failure, occurring due to hypobaric hypoxia-anoxia-induced erythropoiesis reported in both native mountain residents and new climbers after prolonged stays at high altitudes. CMS can be accelerated by smokinginduced carboxyhemoglobinemia at high altitudes. The core pathology of polycythemia is both a boon to mountain adaptation and athletic training as well as a bane towards high altitude deterioration (HAD) leading to CMS. 1,2 A large number of explorers and adventurers along with 140 million people residing at altitudes above 3000 m/9800 ft are at risk of contracting altitude illness. CMS along with hypoxia, cold, and other physiological extreme altitude stressors can contribute to lassitude, cyanosis, thromboembolic phenomena, right ventricular enlargement, myocardial infarction, cerebral infarction, stroke, and frostbite. CMS can be asymptomatic or oligosymptomatic at extreme altitudes above 5500 m/18 000 ft and may be confused with high altitude cerebral edema (HACE). The case being discussed highlights some important aspects of an insidious presentation of CMS which was confused with HACE at an extreme altitude of 6400 m/21 000 ft in the Karakoram Himalayas. Case Presentation A 26-year-old healthy non-smoking, non-drinking explorer from plains country underwent an extended staged-graded acclimatization schedule before reaching 6400 m/21 000 ft. After an uneventful stay of 70 days at that altitude, he reported moderate to severe occipital headache, breathlessness, and two episodes of vomiting to the medical doctor staying at 5790 m/19 000 ft in November. Though there was no history of gain in altitude or exertion, altitude illness was suspected. Rest, plenty of fluids, acetaminophen 500 mg, acetazolamide 250 mg and intravenous dexamethasone 8 mg were initiated following which the patient reported mild improvement. He continued to have headache, breathlessness, and nausea the http://ijtmgh.com