Temporal variability in the occurrence of the most extreme snowfall years, both those with abundant snowfall amounts and those lacking snowfall, was examined using a set of 440 quality-controlled, homogenous U. S. snowfall records. The frequencies with which winter-centered annual snowfall totals exceeded the 90th and 10th percentile thresholds at individual stations were calculated from 1900-01 to 2006-07 for the conterminous United States, and for 9 standard climate regions. The area-weighted conterminous U. S. results do not show a statistically significant trend in the occurrence of either high or low snowfall years for the 107-yr period, but there are regional trends. Large decreases in the frequency of low-extreme snowfall years in the west north-central and east north-central United States are balanced by large increases in the frequency of low-extreme snowfall years in the Northeast, Southeast, and Northwest. During the latter portion of the period, from 1950-51 to 2006-07, trends are much more consistent, with the United States as a whole and the central and northwest U. S. regions in particular showing significant declines in high-extreme snowfall years, and four regions showing significant increases in the frequency of low-extreme snowfall years (i.e., Northeast, Southeast, south, and Northwest). In almost all regions of the United States, temperature during November-March is more highly correlated than precipitation to the occurrence of extreme snowfall years. El Nino events are strongly associated with an increase in low-extreme snowfall years over the United States as a whole, and in the northwest, northeast, and central regions. A reduction in low-extreme snowfall years in the Southwest is also associated with El Nino. The impacts of La Nina events are strongest in the south and Southeast, favoring fewer high-extreme snowfall years, and, in the case of the south, more low-extreme snowfall years occur. The Northwest also has a significant reduction in the chance of a low-extreme snowfall year during La Nina. A combination of trends in temperature in the United States and changes in the frequency of ENSO modes influences the frequency of extreme snowfall years in the United States.