The frequency and intensity of extreme high temperature (EHT) in the Northern Hemisphere exhibit remarkable low-frequency (LF) variations (longer than 10 years) in summer during 1951–2017. Five hotspots featuring large LF variations in EHT were identified, including western North America–Mexico, eastern Siberia, Europe, central Asia, and the Mongolian Plateau. The probability density functions show that the higher EHT occurrences over these hotspots in recent decades is consistent with the shifted average and increased variances in daily mean temperature. The common features of the LF variation in EHT frequency over all domains are the remarkable increasing trends and evident decadal to multidecadal variations. The component of decadal to multidecadal variations is the main contribution to the LF variations of temperature in the last century. Further analysis shows that the coherent variability of decadal to multidecadal temperature variations over western North America–Mexico, eastern Siberia, Europe, and the Mongolian Plateau are the footprints of a dominant natural internal signal: the Atlantic multidecadal oscillation. It contributes to the variations in temperature over these hotspots via barotropic circumglobal teleconnection, which imposes striking anomalous pressure over these regions. This study implies that natural internal variability plays an important role in making hotspots more vulnerable to EHT.