Elizabeth Kostova was born Elizabeth Z. Johnson in New London, Connecticut and raised in Knoxville, Tennessee where she graduated from the Webb School of Knoxville. She received her undergraduate degree from Yale University and a Master of Fine Arts from the University of Michigan, where she won the 2003 Hopwood Award for her Novel-in-Progress. She is married to a Bulgarian scholar and has taken his family name.

Her first novel, The Historian, was published in 2005 and it has become a best-seller.

In May 2007, the Elizabeth Kostova Foundation was created. The Foundation helps support Bulgarian creative writing, the translation of contemporary Bulgarian literature into English, and friendship between Bulgarian authors and American and British authors.

Kostova released her second novel The Swan Thieves on January 12, 2010. Her third novel, The Shadow Land, was released in 2017.