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Atherton began riding BMX at the age of 8 and mountain biking at the age of 11. She was both Sunday Times' Sportswoman of the Year and BBC Midlands Junior Sportswoman of the Year in 2005, and then BBC Midlands Sportswoman of the Year in 2008. In October 2015, a video of Atherton overtaking 91 competitors in five minutes during a race went viral.
Since 2007, Atherton has been part of the Animal Commençal race team along with brothers Dan Atherton and Gee Atherton. In 2012, Atherton, along with her brothers Dan, Gee and Marc Beaumont, signed with GT Bicycles. In 2015, Atherton and her brothers signed with Trek Bicycle Corporation to race for Trek Atherton Racing.
Atherton was involved in a collision with a pickup truck whilst on a time trial training ride with her brothers Dan and Gee in Santa Cruz, California, on 18 January 2009. She sustained a dislocated shoulder which, after later needing a nerve graft, ruled her out of the 2009 racing season, including the September World Championships in Canberra.
In September 2012, Atherton took the final World Cup round on a diverse and testing Norwegian track and clinched the overall title, despite missing the opening race of the season. The Norwegian race was the final round of a seven-round series in which Atherton claimed a win in 5 of the 6 events she raced.
In June 2016, Atherton became the first woman to win 10 consecutive rounds of the World Cup, surpassing the record previously held by Anne-Caroline Chausson. In September that same year, she also achieved the unprecedented feat of winning every round in a World Cup season.