The lovable Miss Ellie, whose lopsided tongue always stuck out of the side of her mouth, died on Tuesday in her Tennessee home, the local Mountain Press daily newspaper said.
Rescued by owner Dawn Goehring when she was seven years old, Miss Ellie, who drew smiles with the shock of blonde hair above her eyes, had helped raise over US$100,000 for charity.
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Despite being blind in her final years, she won Animal Planet's 'World's Ugliest Dog' award last year.
Animal shelter plan
She also starred in Animal Planet's 'Dog 101' and was featured in numerous commercials and television programs.
Miss Ellie had been set to travel to California this month to defend her title.
She was part of a troupe of dogs that Goehring trained and which performed regularly at the Comedy Barn in Pigeon Forge.
Goehring said she is now planning on raising money to open a new animal shelter.
"I am going to make that my driven goal to do in her honor," she told the Mountain Press.
