The exhibition, Leonardo de Vinci: Anatomist, will be held in the Queen's Gallery of Buckingham Palace in May.
On show will be the contents of the 16th Century Leoni binding, which held the drawings until around 1900.
"The binding was effectively the tomb of the drawings...It ensured that they were not circulated or published" the BBC quoted curator Martin Clayton as saying.
"Only around 1900 did they emerge from the binding, and we now know that they were among the most amazingly detailed and accurate anatomical drawings of all time.
"And this exhibition will be the greatest opportunity since Leonardo's death to marvel at his achievement," Clayton said.
The Buckingham Palace exhibition follows closely behind the sell-out show of the artist's rare surviving paintings at London's National Gallery.
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Leonardo da Vinci has long been recognised as one of the greatest artists of the Renaissance but the palace exhibition will also show him as highly perceptive anatomist, who was way ahead of his time.
He produced detailed studies of bones, muscles and internal organs, including the heart and the brain.
It is thought he intended to publish his studies but when he died in 1519 they remained among his private papers.
Leonardo left his notebooks and drawings to his assistant Francesco Melzi, whose son sold them to the sculptor Pompeo Leoni.
Leoni had the anatomical drawings bound together in an album with his name alongside Leonardo's in gold lettering on the cover: Disegni di Leonardo da Vinci restaurati da Pompeo Leoni - "Drawings by Leonardo da Vinci, preserved by Pompeo Leoni".