This review aims to update our understanding of human cold adaptation. First, an overview of the thermoregulatory response to cold is provided, with some recent updates in human brown adipose tissue (BAT). Variation in BAT activity and multiorgan contributions to cold-induced thermogenesis were introduced. We found that individuals with less BAT activity rely more on shivering to compensate for less non-shivering thermogenesis (NST). The mechanisms of cold-induced vasoconstriction are summarized, including the role of arteriovenous anastomoses, adrenergic neural function, and inhibition of the nitric oxide vasodilator pathway. In addition, cold-induced vasodilation (CIVD) during cold immersion of the distal extremities is summarized with some recent updates in physiological mechanism. Furthermore, the cold shock response at the onset of cold immersion is introduced. Next, categorization of cold acclimatization/acclimation into habituation of shivering and metabolic and insulative adaptation are provided, with some recent updates. Especially, the rediscovery of human BAT has clarified metabolic acclimation, where increased NST replace shivering. Then, a greater CIVD response in populations in cold regions has been reported, whereas recent laboratory studies suggest no increase in CIVD after repeated cold exposure. To prevent cold injuries, individuals should not rely on habituation through repeated cold exposure. In addition, habituation to the cold shock response after repeated cold water immersion could help reduce the number of drownings. Furthermore, cross-adaptation between cold and nonthermal factors in the thermoregulatory response is summarized. Recent studies explored the relationship between exercise training and BAT activity, although this remains unresolved, depending on the exercise intensity and environmental conditions. The effects of exercise with cold exposure on the thermoregulatory response to cold are summarized in studies including divers working in cold water. We investigated the effect of exercise training in cold water, which resulted in increased muscle deoxygenation during submaximal exercise and greater anerobic power. Moreover, the effects of a hypoxic environment on cold adaptation are summarized. Elevated basal metabolism and higher distal skin temperature in highlanders could improve their cold tolerance. Finally, factors affecting cold adaptation are discussed. The type of cold adaptation may depend on the specific thermoregulatory responses repeated during the adaptation process.