The dog, named Toad, arrived as a stray at the Oklahoma City Animal Welfare shelter where her second mouth was discovered.
From there, she soon made her way to Mutt Misfits, a nearby rescue centre for animals with major medical issues, where she remains still.
Heather Hernandez, the charity's founder and Toad's owner, said: "Immediately we recognised that she was very different to most dogs.
"She was initially showing lots of aggression but once she and I got together, that all disappeared.
"Upon her initial intake exam at the shelter, our veterinarian originally thought she had extra ears.
"But once we sedated her for her spay surgery, that's when we discovered that she actually only has one ear and two mouths!"
'TOAD'S LIFE IS MOSTLY NORMAL'
Toad's second mouth rarely causes the pooch pain, her owners claim.
Heather added: "We originally thought it had two teeth but we did a dental cleaning on the ear a few weeks ago and discovered it has several.
"A few of the ear teeth had cracked, which caused some discomfort, but once we removed the broken teeth she was good as new.
"The main teeth we kept since they appear to be attached to the skull.
"We certainly can't rule out that the ear mouth might cause further issues in the future but that's okay, we can just take it one day at a time."
All of the other dogs move out of the way for her, everyone has accepted that she gets special attention because our bond is so tight.
The second mouth can't actually function as a mouth, however.
"It has what looks like lips in which the opening of the lips is the part that actively salivates," said Heather.
"The ear mouth does not have a jaw though so it doesn't actually open and close. The skin and ear lips are what move primarily."
And while the five-year-old pooch does have a few other medical challenges, Heather says Toad's life is mostly normal.
"She doesn't hear, see or smell very well because everything in her head is misaligned, but otherwise she's totally normal," said Heather.
"She is very devoted and loyal to her family. She thinks she's a lap dog and always insists on sitting on my lap.
"All of the other dogs move out of the way for her, everyone has accepted that she gets special attention because our bond is so tight."
Other mainstays at Mutt Misfits include Scary Jack, who owes his unusual appearance to a severe case of puppy strangles.
Heather said: "The charity was formed because in shelter settings there aren't many organisations that specialise in the special needs pups.
"Most rescue groups take in highly adoptable pups from shelters and re-home them. We focus on animals with major medical issues and saving the sick and injured pets.
"We like to help people to understand that animals don't have to be perfect to make perfect pets."
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