The Oscar-winning filmmaker will receive the coveted Golden Lion award at the 70th annual event, where a restored version of his 1977 thriller Sorcerer will be screened.
The event will be doubly special for Friedkin as he will celebrate his 78th birthday during the festival on August 29 (13).
He says, "Venice, especially during the film festival, is a spiritual home to me. The Golden Lion is something I never expected but am proud to accept it with gratitude and love."
Friedkin follows in the footsteps of previous Golden Lion recipients, including John Woo, Tim Burton, David Lynch, Clint Eastwood, Stanley Kubrick and Steven Spielberg.
The 2013 festival will kick off on 28 August (13) and runs until 7 September (13).