The rare but devastating infection is found in warm freshwater places, like lakes, rivers and hot springs.
While the chances of getting it are slim, so are the chances of surviving it if you do.
Here are some things to keep in mind when swimming during the summer months.
The infection is caused by tiny amoebas, single-celled organisms, that live in freshwater.
In the United States, most of the infections occur in southern states, where the water is warmer year round.
The amoebas thrive in warm water, even in temperatures up to 115 degrees.
The disease infects people when water containing the amoebas enters the body through the nose and travels to the brain where it destroys tissue.
You can't be infected by drinking contaminated water.
The amoebas can't live in saltwater or properly treated pool or hot tub water.
The initial symptoms may include headache, fever, nausea or vomiting.
As the disease progresses, the symptoms can include a stiff neck, general confusion, loss of balance, seizures and hallucinations.
Once the symptoms begin, the disease develops rapidly and usually causes death within about five days.
One thing to remember, the risk is very low.
There have only been 40 reported infections in the United States during the decade from 2007 to 2016, even though millions of people swim in freshwater each year.
To protect yourself, use a nose clip when swimming. That will keep any amoebas from making their way up your nasal passages to your brain.