Lewis, 85, who starred in more than 45 films in a career spanning five decades, made the announcement in a statement posted by the Muscular Dystrophy Association (MDA), on its website.
"As a labor of love, I've hosted the annual telethon since 1966, and I'll be making my final appearance on the show this year by performing my signature song, "You'll Never Walk Alone,'" he said in the statement.
Lewis has turned the ballad from the Rodgers and Hammerstein musical "Carousel" into an anthem for MDA by performing it at the conclusion of each telethon over the years.
Lewis did not say why he was retiring at this stage, but an MDA spokesman said the comedian's departure coincided with a change in the program's format from a 21 1/2-hour telethon to a six-hour broadcast centered around prime time.
"We needed to change the format for our show, and we're creating a new telethon era now," the spokesman, Jim Brown, told Reuters.
"We're very, very thankful that Jerry Lewis is performing 'You'll Never Walk Alone,'" he said. "He is a world-class humanitarian, and no-one rivals him in terms of his commitment to the cause."
Lewis said he would continue to serve as national MDA chairman, adding, "I'll never desert MDA and my kids."
The program has raised in excess of $2 billion for the fight against muscular dystrophy through 900 hours of live broadcasts over the years, and has become an annual Labor Day weekend institution on television.
Your Los Angeles Media Connection LAMediaWatch.com
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