Elwood, the Chihuahua and Chinese Crested cross whose unusual appearance won him the 2007 title of world's ugliest dog, has died at the age of eight.
"It was sudden," said owner Karen Quigley, 52, who adopted Elwood in 2006. "He was in my arms."
The 2.3kg (5lb) dog died on Thanksgiving morning last Thursday at his home in the US state of New Jersey.
Quigley said her vet believed he might have had some form of cancer.
He had been dealing with some heath issues in recent months but appeared to be doing well.
Elwood was dark and nearly hairless, except for a tuft of white hair on his forehead, and had a long, protruding tongue.
A breeder who owned Elwood originally said he was too ugly to sell and was planning to put him down.
Karen Quigley with Elwood after winning world's ugliest dog contest in Petaluma
But he was taken in by one of the breeder's friends and Karen Quigley adopted him when he was nine months old.
After winning the annual world's ugliest dog contest at the Marin-Sonoma County Fair in 2007, fans from around the world sent him letters and postcards and he was often referred to as Yoda or E.T.
Elwood appeared at more than 200 charity events before his retirement earlier this year.
After adopting him, owner Karen Quigley wrote Everyone Loves Elwood, a children's book about self-acceptance.
"Elwood had a magical power to make people smile and laugh that made him very attractive, especially to children," said Quigley.
"He would pose for photos with people at charitable events.
"He was the most beautiful dog I've ever seen. He taught children it's OK to be different."
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