Luke Humphries stunned by Gian van Veen in World Matchplay opener
Humphries was hoping to become just the fourth man to defend his title in Blackpool after he won for the first time in 2024.
But he was not at his best at the Winter Gardens and was downed 10-8 by the Dutchman van Veen, who celebrated the best win of his career.
Humphries' exit will make Luke Littler the overwhelming favourite for the event, with the teenager kicking off his campaign against Ryan Searle on Sunday.
Despite not being at his best, Humphries still had chances to beat Van Veen but could only convert 28 per cent of his double attempts.
Van Veen made his move to break at 7-7 and then eventually got over the finishing line on his fourth match dart.
'This feels like the biggest win of my career, to be here at the iconic Winter Gardens,' he said.'Finally I got that winning double and this is the biggest win of my career.
'I guess this makes me the number one seed! I am just going to enjoy this one tonight.
'It means the world, I felt really relaxed on the stage, to beat the reigning champion and world number one, it's an amazing feeling.
'Luke is a true champion, a great guy and the true world number one. To beat the world number one is amazing but it is just one game. I take it game by game.'
James Wade celebrated 20 successive years at the World Matchplay by coasting to a 10-3 win over Joe Cullen.
Wade threw his best-ever average at the Winter Gardens of 104.44, hitting 10 out of 15 doubles.
He hit out at anyone who believes he cannot win the title next week, referencing an old spat with Sky Sports pundit Wayne Mardle who once questioned him.
'I can do it again, 100 per cent. I'm good enough to win this tournament. I mean, I think if anyone believes any different, I think they're a little bit Wayne Mardle-d.
'But I respect Wayne as well. I mean, Wayne's a wonderful person. He's got great knowledge of the game. I think he writes quite a few other players off.
'But I can win this tournament. Of course, again, it's no secret.'
Danny Noppert beat Cameron Menzies 10-2, setting up a second-round meeting with Van Veen.
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