The Antarctica and High Arctic regions are extreme environments, with average maximum temperatures below 0 °C for most days of the year. Interestingly, fungi inhabit these regions. This review describes the history of fungal surveys near the Syowa Station and the fungal diversity in this region. In the High Arctic region, I summarize the changes in the fungal communities of the glacial retreat areas of Ny-Ålesund, Norway and Ellesmere Island, Canada, in response to climate change. In addition, the ability of Antarctic and Arctic fungi to secrete enzymes at sub-zero temperatures is presented. Finally, the future directions of Antarctic and Arctic fungal research are provided.