A lone surfer has died after being attacked by a large shark in the world famous Margaret River surfing region.

Police and the RAC helicopter were dispatched to a beach south of Cowaramup Bay in the Gracetown area this morning.

Police have confirmed the man has died from his injuries after being rushed to hospital. They could not say how old he was or confirm his identity.

The man is believed to have been attacked while surfing on his own, at a beach only accessible by four-wheel-drive vehicles.

The attack reportedly happened about 8.15am. He was then rushed to Margaret River Hospital with serious injuries.

The Gracetown area is a well-known and notorious surfing spot. A popular local surfer Brad Smith, 29, was killed by a shark while surfing at Lefthanders beach in July, 2004.

There were reports Mr Smith was attacked by two sharks, described as being great whites, but the type of species was never confirmed.

A teenager surfer, Cameron Rowe, saw the two sharks and described how one kept attacking Mr Smith, with blood spilling everywhere.

Western Australia's south-west coast is notorious for sharks, who follow migrating whales in search of food. There have been many white pointer sightings in the area.