The story begins with the unnamed narrator arriving at the house of his friend, Roderick Usher, having received a letter from him in a distant part of the country complaining of an illness and asking for his help. As he arrives, the narrator notes a thin crack extending from the roof, down the front of the building and into the lake.

It is revealed that Roderick’s twin sister, Madeline, is also ill and falls into cataleptic, deathlike trances. The narrator is impressed with Roderick’s paintings, and attempts to cheer him by reading with him and listening to his improvised musical compositions on the guitar. Roderick sings “The Haunted Palace”, then tells the narrator that he believes the house he lives in to be alive, and that this sentience arises from the arrangement of the masonry and vegetation surrounding it.